Pigmentation Disorders

More than 1.8 billion individuals utilize social media, a number that continues to grow as the social media market expands.¹ Social media enables individuals, groups, and organizations to efficiently disperse and access information^2-4 and also provides a structure that encourages collaboration between patients, staff, and physicians that cannot be achieved by other communication modalities.^4-6 Expert opinions and related educational materials can be shared globally, improving collaboration between dermatologists.⁶ A structured social networking site for sharing training materials, research, and ideas can help bring the national dermatology community together in a new way.

Other professions have employed social networking tools to accomplish similar goals of organizing training resources; radiology has an electronic database that allows sharing of training materials and incorporates social networking capabilities.⁷ Their Web software provides functionality for individual file uploading and supports collaboration and sharing, all while maintaining the security of uploaded information. General surgery has already addressed similar concerns via a task force that incorporates all the essential organizations in surgical education.⁸ Increased satisfaction and academic abilities have been demonstrated with their collaborative curriculum.⁹ Gastroenterologists also utilize electronic resources; one study showed that using videos to educate patients prior to colonoscopies was superior to face-to-face education.¹⁰ In addition, video education may free up time for office staff to accomplish other tasks.

As a specialty, dermatology has not been a leader in the implementation of social networking for collaboration and training purposes. Every dermatologist is an educator. To maintain a successful practice, dermatologists must keep up-to-date on their own clinical knowledge, provide training to their staff, and educate their patients. Although there are numerous educational resources available to dermatologists, an informal survey of 30 dermatology faculty members revealed a practice gap in awareness and utilization of these expanding electronic resources.¹¹

To better understand the needs of the specialty as a whole, we chose to focus on one aspect of dermatology education: resident training. The goal of our study was to survey dermatology residents and faculty to gain a better understanding of how they currently provide education and what online resources and social networking sites they currently use or would be willing to use. The study included 3 central hypotheses: First, residents would be less satisfied with their current curriculum and residents would report greater contributions to the curriculum relative to faculty. Second, both residents and faculty of smaller programs would be more interested in collaborative educational resources relative to larger programs. Lastly, residents would be more willing than faculty to participate in social networking for educational purposes.

Methods

This study was granted institutional review board exemption. Two surveys were developed by the authors to assess the current structure and satisfaction of dermatology residency curriculum and the willingness to participate in social networking to use and share educational materials. The surveys were evaluated for relevance by the survey evaluation team of the Association of Professors of Dermatology (APD). The instrument was not pilot tested.

The surveys were electronically distributed using an online service to dermatology faculty via the APD listserve, which comprised the entirety of the APD membership in 2014. The resident survey was distributed to the dermatology residents via the American Society for Dermatologic Surgery listserve, which included all residents in training (2013-2014 academic year). Second and third invitations to complete the surveys were distributed 3 and 5 weeks later, respectively.

Resident and faculty responses were compared. Additionally, responses were stratified for large (>9 residents) and small programs (≤9 residents) for comparison. Descriptive statistics including means and medians for continuous variables and frequency tables for categorical variables were generated using research and spreadsheet software.

Results

There were 137 survey respondents; 52 of 426 (12.2%) dermatology faculty and 85 of 1539 (5.5%) dermatology residents responded to the survey. Small programs accounted for 24% of total survey responses and 76% were from large programs.

Current Curriculum

The majority of dermatology faculty (44%) and residents (35%) identified 1 to 2 faculty members as contributing to the creation and organization of their respective curricula; however, a notable percentage of residents (9%) reported that no faculty contributed to the organization of the curriculum. Residents noted that senior residents carry twice the responsibility for structuring the curriculum compared to faculty (61% vs 32% of the workload), but faculty described an even split between senior residents and faculty (47% vs 49% of the workload). Faculty believed their residents spend a similar amount of time in resident- and faculty-led instruction (38% vs 35% of their time); however, the majority of residents reported spending too little time in faculty-led instruction (53%). When residents ranked their preference for learning modes, faculty-led and self-study learning were ranked first and second by 48% and 45% of residents, respectively. Resident-led instruction was ranked last by 66% of residents. Likewise, a majority of residents (53%) described their amount of time in faculty-led instruction as too little.

When asked what subjects in dermatology were lacking at their programs, residents reported clinical trials (47%), skin of color (46%), cosmetic dermatology (34%), and aggressive skin cancer/multidisciplinary tumor board (32%). Although 11% of residents reported lacking inpatient dermatology in their curriculum, 0% of faculty reported the same. A notable percentage of faculty reported nothing was lacking compared to residents (25% vs 7%). Despite these different views between residents and faculty on their contributions to and structure of their curriculums, both faculty and residents claimed overall satisfaction (satisfied or very satisfied) with their program’s ability to optimally cover the field of dermatology in 3 years (100% and 91%, respectively).

Large Versus Small Residency Programs

When stratifying the resident responses for small versus large programs, both program sizes reported more time in resident-led instruction than faculty-led instruction. Likewise, residents in both program sizes equally preferred self-study or faculty-led instruction to resident-led instruction. Residents at small programs more often reported lacking instruction in rheumatology, immunobullous diseases, and basic science/skin biology compared to large-program residents. Compared to large-program faculty, small-program faculty reported lacking instruction in cosmetic dermatology.

Faculty at small programs reported spending too little time preparing for their faculty-led instruction compared to faculty at large programs (44% vs 12%). All (100%) of the faculty at small programs were likely to seek out study materials shared by top educators, while 77% of faculty at large programs were likely to do the same. When asked if faculty would translate what their program does well into an electronic format for sharing, 30% of large-program faculty were likely to do so compared to 11% of small-program faculty (Figure 1).

Use of Online Educational Materials and Interest in Collaboration

A majority of faculty and residents stated that they use online educational materials as supplements to traditional classroom lecture and print materials (81% vs 86%); however, almost twice as many residents stated that online educational materials were essential to their current study routines compared to faculty (39% vs 21%).

The majority of faculty (92%) and residents (84%) were either interested or very interested in a collaborative online curriculum. Both residents (85%) and faculty (81%) stated they would be likely to seek out online educational materials shared by top educators. Although both residents and faculty reported many aspects of their curriculums they thought could be beneficial to other dermatology programs (Table 1), only 27% of faculty and 19% of residents were likely to translate those strengths into a shareable electronic format. Several reasons were reported for not contributing to an online curriculum, with lack of time being the most common reason (Table 2).

Eighty percent of residents and 88% of faculty reported they were either interested or very interested in being more connected/interactive with their dermatology peers nationally (Figure 2). Likewise, 94% of residents and 87% of faculty agreed that the dermatology community could benefit from a social networking site for educational collaboration. Four times as many residents versus faculty currently use social networking sites (eg, Facebook, LinkedIn, Google Groups) as a primary mode of communication with distant professional peers. The majority of residents (52%) reported they would be likely to participate in a professional social networking site, while the majority of faculty (50%) stated they were neutral on their likelihood of participating. Both residents and faculty reported lack of time as a common reason for being unlikely to utilize a professional social networking site. Other barriers to participation are listed in Table 3.

Comment

This study showed how dermatology faculty and residents currently provide training and what online resources and social networking sites they currently use or would be willing to use. The generalizability of the conclusions is limited by the low response rate for the surveys. The results demonstrated the different views between faculty and residents and between large and small residency programs on various topics. This microcosm of dermatology training can likely be applied to other training scenarios in dermatology, including patient education; training of nurses, physician extenders, and office staff; continuing medical education for physicians; and peer-to-peer collaboration.

Hypothesis 1: Partially Proven

We hypothesized that residents would report less satisfaction with their current curriculum and would report greater resident contributions to the curriculum relative to faculty. Overall, residents and faculty reported satisfaction with their curriculums to provide up-to-date information and breadth in the field of dermatology. Despite their overall satisfaction, more residents reported lacking instruction in several dermatology subtopics compared to faculty. Additionally, residents believed they spend twice as much time structuring their curriculum compared to faculty, with some residents reporting no faculty involvement. Although residents preferred faculty-led instruction, a majority of residents reported they do not have enough faculty-led didactics. The preference for faculty-led training is likely due to the expertise of faculty compared to residents.

Hypothesis 2: Partially Proven

We also hypothesized that both residents and faculty of smaller programs would be more interested in collaborative educational resources relative to larger programs. Although there was no difference in interest between residents at small versus large programs, there was a difference between faculty at small versus large programs. Small-program faculty were more interested in using shared materials than larger programs, while large-program faculty were more likely to share their educational materials. Small-program faculty reported spending too little time preparing their lectures, which is possibly due to a lack of time for preparation. Additionally, residents and faculty at smaller programs report their curriculum was lacking specific dermatology topics compared to large programs. These disparities between program sizes indicate a need for a social networking site for training collaboration in dermatology. Large programs have the ability to share what they do well, which small programs are eager to utilize.

Hypothesis 3: Not Proven

We hypothesized that residents would be more willing than faculty to participate in social networking for educational purposes. The majority of faculty and residents were interested in participating in a collaborative online curriculum and using the shared materials from top educators; however, even though such large majorities favored collaboration and sharing, only 27% of faculty and 19% of residents were likely to translate their own materials into a shareable format. Although lack of time was the most common reason for not sharing materials, electronic methods may have the potential to ultimately save time and remove the burden of content creation. The time it would take to translate selected personal training materials into a shareable form would be made up for by the time saved using another educators’ materials. Updating and customizing shared online educational materials can be much quicker and easier than educators creating materials on their own. Dermatologists would be more efficient facilitators of training via high-quality shared materials while decreasing the time burden associated with resident education.⁵ Another concern for not sharing or participating in a social networking site was skepticism of information security on such a network. The poor organization and information overload of online resources can compound the already existing time constraints on dermatologists, which may limit their ability to utilize such valuable resources. In addition, quality of online resources is not always guaranteed, and determining the sources that are high quality is sometimes a difficult task.⁶ For online materials to remain useful, there should be a peer-review process to evaluate quality and assess satisfaction.⁵

Solution: Create a Dermatology Task Force

A dermatology task force could facilitate the resolution of these challenges of online materials. In addition, a task force could cover the administrative support needed to ensure security and provide maintenance on social networks.

The main limitation to implementing a social network is the presence of the administrative infrastructure to jumpstart its creation. A task force incorporating the essential stakeholders in dermatology training is the first step. With inclusive representation from all of the smaller professional dermatology societies, the American Academy of Dermatology is optimally positioned to create this task force. With existing information technologies, a task force could address the concerns revealed in our survey as well as any future concerns that may arise.

The goal is a single social network for dermatologists that has the capability of improving communication and collaboration between professional peers regardless of their practice setting. Such a network is ideal for the practicing dermatologist for the purposes of staff training, patient education, and obtaining continuing medical education credit. Additionally, peer group collaboration would facilitate the understanding and completion of the evolving requirements for Maintenance of Certification from the American Board of Dermatology. The availability of quality shared materials would save time and increase efficiency of an entire dermatology practice. Materials that aid in patient education would allow office staff to dedicate their time to other tasks, thereby increasing productivity. Shared training materials would decrease the burden of staff education, providing more time for advanced hands-on training. This method of collaborative effort is capable of advancing the field of dermatology as a whole. It can overcome geographical and institutional barriers to connect dermatologists with similar interests worldwide; disseminate advances in diagnosis and treatment; and improve the quality of dermatology training of dermatologists, staff, and patients.

More than 1.8 billion individuals utilize social media, a number that continues to grow as the social media market expands.¹ Social media enables individuals, groups, and organizations to efficiently disperse and access information^2-4 and also provides a structure that encourages collaboration between patients, staff, and physicians that cannot be achieved by other communication modalities.^4-6 Expert opinions and related educational materials can be shared globally, improving collaboration between dermatologists.⁶ A structured social networking site for sharing training materials, research, and ideas can help bring the national dermatology community together in a new way.

Other professions have employed social networking tools to accomplish similar goals of organizing training resources; radiology has an electronic database that allows sharing of training materials and incorporates social networking capabilities.⁷ Their Web software provides functionality for individual file uploading and supports collaboration and sharing, all while maintaining the security of uploaded information. General surgery has already addressed similar concerns via a task force that incorporates all the essential organizations in surgical education.⁸ Increased satisfaction and academic abilities have been demonstrated with their collaborative curriculum.⁹ Gastroenterologists also utilize electronic resources; one study showed that using videos to educate patients prior to colonoscopies was superior to face-to-face education.¹⁰ In addition, video education may free up time for office staff to accomplish other tasks.

As a specialty, dermatology has not been a leader in the implementation of social networking for collaboration and training purposes. Every dermatologist is an educator. To maintain a successful practice, dermatologists must keep up-to-date on their own clinical knowledge, provide training to their staff, and educate their patients. Although there are numerous educational resources available to dermatologists, an informal survey of 30 dermatology faculty members revealed a practice gap in awareness and utilization of these expanding electronic resources.¹¹

To better understand the needs of the specialty as a whole, we chose to focus on one aspect of dermatology education: resident training. The goal of our study was to survey dermatology residents and faculty to gain a better understanding of how they currently provide education and what online resources and social networking sites they currently use or would be willing to use. The study included 3 central hypotheses: First, residents would be less satisfied with their current curriculum and residents would report greater contributions to the curriculum relative to faculty. Second, both residents and faculty of smaller programs would be more interested in collaborative educational resources relative to larger programs. Lastly, residents would be more willing than faculty to participate in social networking for educational purposes.

Methods

This study was granted institutional review board exemption. Two surveys were developed by the authors to assess the current structure and satisfaction of dermatology residency curriculum and the willingness to participate in social networking to use and share educational materials. The surveys were evaluated for relevance by the survey evaluation team of the Association of Professors of Dermatology (APD). The instrument was not pilot tested.

The surveys were electronically distributed using an online service to dermatology faculty via the APD listserve, which comprised the entirety of the APD membership in 2014. The resident survey was distributed to the dermatology residents via the American Society for Dermatologic Surgery listserve, which included all residents in training (2013-2014 academic year). Second and third invitations to complete the surveys were distributed 3 and 5 weeks later, respectively.

Resident and faculty responses were compared. Additionally, responses were stratified for large (>9 residents) and small programs (≤9 residents) for comparison. Descriptive statistics including means and medians for continuous variables and frequency tables for categorical variables were generated using research and spreadsheet software.

Results

There were 137 survey respondents; 52 of 426 (12.2%) dermatology faculty and 85 of 1539 (5.5%) dermatology residents responded to the survey. Small programs accounted for 24% of total survey responses and 76% were from large programs.

Current Curriculum

The majority of dermatology faculty (44%) and residents (35%) identified 1 to 2 faculty members as contributing to the creation and organization of their respective curricula; however, a notable percentage of residents (9%) reported that no faculty contributed to the organization of the curriculum. Residents noted that senior residents carry twice the responsibility for structuring the curriculum compared to faculty (61% vs 32% of the workload), but faculty described an even split between senior residents and faculty (47% vs 49% of the workload). Faculty believed their residents spend a similar amount of time in resident- and faculty-led instruction (38% vs 35% of their time); however, the majority of residents reported spending too little time in faculty-led instruction (53%). When residents ranked their preference for learning modes, faculty-led and self-study learning were ranked first and second by 48% and 45% of residents, respectively. Resident-led instruction was ranked last by 66% of residents. Likewise, a majority of residents (53%) described their amount of time in faculty-led instruction as too little.

When asked what subjects in dermatology were lacking at their programs, residents reported clinical trials (47%), skin of color (46%), cosmetic dermatology (34%), and aggressive skin cancer/multidisciplinary tumor board (32%). Although 11% of residents reported lacking inpatient dermatology in their curriculum, 0% of faculty reported the same. A notable percentage of faculty reported nothing was lacking compared to residents (25% vs 7%). Despite these different views between residents and faculty on their contributions to and structure of their curriculums, both faculty and residents claimed overall satisfaction (satisfied or very satisfied) with their program’s ability to optimally cover the field of dermatology in 3 years (100% and 91%, respectively).

Large Versus Small Residency Programs

When stratifying the resident responses for small versus large programs, both program sizes reported more time in resident-led instruction than faculty-led instruction. Likewise, residents in both program sizes equally preferred self-study or faculty-led instruction to resident-led instruction. Residents at small programs more often reported lacking instruction in rheumatology, immunobullous diseases, and basic science/skin biology compared to large-program residents. Compared to large-program faculty, small-program faculty reported lacking instruction in cosmetic dermatology.

Faculty at small programs reported spending too little time preparing for their faculty-led instruction compared to faculty at large programs (44% vs 12%). All (100%) of the faculty at small programs were likely to seek out study materials shared by top educators, while 77% of faculty at large programs were likely to do the same. When asked if faculty would translate what their program does well into an electronic format for sharing, 30% of large-program faculty were likely to do so compared to 11% of small-program faculty (Figure 1).

Use of Online Educational Materials and Interest in Collaboration

A majority of faculty and residents stated that they use online educational materials as supplements to traditional classroom lecture and print materials (81% vs 86%); however, almost twice as many residents stated that online educational materials were essential to their current study routines compared to faculty (39% vs 21%).

The majority of faculty (92%) and residents (84%) were either interested or very interested in a collaborative online curriculum. Both residents (85%) and faculty (81%) stated they would be likely to seek out online educational materials shared by top educators. Although both residents and faculty reported many aspects of their curriculums they thought could be beneficial to other dermatology programs (Table 1), only 27% of faculty and 19% of residents were likely to translate those strengths into a shareable electronic format. Several reasons were reported for not contributing to an online curriculum, with lack of time being the most common reason (Table 2).

Eighty percent of residents and 88% of faculty reported they were either interested or very interested in being more connected/interactive with their dermatology peers nationally (Figure 2). Likewise, 94% of residents and 87% of faculty agreed that the dermatology community could benefit from a social networking site for educational collaboration. Four times as many residents versus faculty currently use social networking sites (eg, Facebook, LinkedIn, Google Groups) as a primary mode of communication with distant professional peers. The majority of residents (52%) reported they would be likely to participate in a professional social networking site, while the majority of faculty (50%) stated they were neutral on their likelihood of participating. Both residents and faculty reported lack of time as a common reason for being unlikely to utilize a professional social networking site. Other barriers to participation are listed in Table 3.

Comment

This study showed how dermatology faculty and residents currently provide training and what online resources and social networking sites they currently use or would be willing to use. The generalizability of the conclusions is limited by the low response rate for the surveys. The results demonstrated the different views between faculty and residents and between large and small residency programs on various topics. This microcosm of dermatology training can likely be applied to other training scenarios in dermatology, including patient education; training of nurses, physician extenders, and office staff; continuing medical education for physicians; and peer-to-peer collaboration.

Hypothesis 1: Partially Proven

We hypothesized that residents would report less satisfaction with their current curriculum and would report greater resident contributions to the curriculum relative to faculty. Overall, residents and faculty reported satisfaction with their curriculums to provide up-to-date information and breadth in the field of dermatology. Despite their overall satisfaction, more residents reported lacking instruction in several dermatology subtopics compared to faculty. Additionally, residents believed they spend twice as much time structuring their curriculum compared to faculty, with some residents reporting no faculty involvement. Although residents preferred faculty-led instruction, a majority of residents reported they do not have enough faculty-led didactics. The preference for faculty-led training is likely due to the expertise of faculty compared to residents.

Hypothesis 2: Partially Proven

We also hypothesized that both residents and faculty of smaller programs would be more interested in collaborative educational resources relative to larger programs. Although there was no difference in interest between residents at small versus large programs, there was a difference between faculty at small versus large programs. Small-program faculty were more interested in using shared materials than larger programs, while large-program faculty were more likely to share their educational materials. Small-program faculty reported spending too little time preparing their lectures, which is possibly due to a lack of time for preparation. Additionally, residents and faculty at smaller programs report their curriculum was lacking specific dermatology topics compared to large programs. These disparities between program sizes indicate a need for a social networking site for training collaboration in dermatology. Large programs have the ability to share what they do well, which small programs are eager to utilize.

Hypothesis 3: Not Proven

We hypothesized that residents would be more willing than faculty to participate in social networking for educational purposes. The majority of faculty and residents were interested in participating in a collaborative online curriculum and using the shared materials from top educators; however, even though such large majorities favored collaboration and sharing, only 27% of faculty and 19% of residents were likely to translate their own materials into a shareable format. Although lack of time was the most common reason for not sharing materials, electronic methods may have the potential to ultimately save time and remove the burden of content creation. The time it would take to translate selected personal training materials into a shareable form would be made up for by the time saved using another educators’ materials. Updating and customizing shared online educational materials can be much quicker and easier than educators creating materials on their own. Dermatologists would be more efficient facilitators of training via high-quality shared materials while decreasing the time burden associated with resident education.⁵ Another concern for not sharing or participating in a social networking site was skepticism of information security on such a network. The poor organization and information overload of online resources can compound the already existing time constraints on dermatologists, which may limit their ability to utilize such valuable resources. In addition, quality of online resources is not always guaranteed, and determining the sources that are high quality is sometimes a difficult task.⁶ For online materials to remain useful, there should be a peer-review process to evaluate quality and assess satisfaction.⁵

Solution: Create a Dermatology Task Force

A dermatology task force could facilitate the resolution of these challenges of online materials. In addition, a task force could cover the administrative support needed to ensure security and provide maintenance on social networks.

The main limitation to implementing a social network is the presence of the administrative infrastructure to jumpstart its creation. A task force incorporating the essential stakeholders in dermatology training is the first step. With inclusive representation from all of the smaller professional dermatology societies, the American Academy of Dermatology is optimally positioned to create this task force. With existing information technologies, a task force could address the concerns revealed in our survey as well as any future concerns that may arise.

The goal is a single social network for dermatologists that has the capability of improving communication and collaboration between professional peers regardless of their practice setting. Such a network is ideal for the practicing dermatologist for the purposes of staff training, patient education, and obtaining continuing medical education credit. Additionally, peer group collaboration would facilitate the understanding and completion of the evolving requirements for Maintenance of Certification from the American Board of Dermatology. The availability of quality shared materials would save time and increase efficiency of an entire dermatology practice. Materials that aid in patient education would allow office staff to dedicate their time to other tasks, thereby increasing productivity. Shared training materials would decrease the burden of staff education, providing more time for advanced hands-on training. This method of collaborative effort is capable of advancing the field of dermatology as a whole. It can overcome geographical and institutional barriers to connect dermatologists with similar interests worldwide; disseminate advances in diagnosis and treatment; and improve the quality of dermatology training of dermatologists, staff, and patients.

More than 1.8 billion individuals utilize social media, a number that continues to grow as the social media market expands.¹ Social media enables individuals, groups, and organizations to efficiently disperse and access information^2-4 and also provides a structure that encourages collaboration between patients, staff, and physicians that cannot be achieved by other communication modalities.^4-6 Expert opinions and related educational materials can be shared globally, improving collaboration between dermatologists.⁶ A structured social networking site for sharing training materials, research, and ideas can help bring the national dermatology community together in a new way.

Other professions have employed social networking tools to accomplish similar goals of organizing training resources; radiology has an electronic database that allows sharing of training materials and incorporates social networking capabilities.⁷ Their Web software provides functionality for individual file uploading and supports collaboration and sharing, all while maintaining the security of uploaded information. General surgery has already addressed similar concerns via a task force that incorporates all the essential organizations in surgical education.⁸ Increased satisfaction and academic abilities have been demonstrated with their collaborative curriculum.⁹ Gastroenterologists also utilize electronic resources; one study showed that using videos to educate patients prior to colonoscopies was superior to face-to-face education.¹⁰ In addition, video education may free up time for office staff to accomplish other tasks.

As a specialty, dermatology has not been a leader in the implementation of social networking for collaboration and training purposes. Every dermatologist is an educator. To maintain a successful practice, dermatologists must keep up-to-date on their own clinical knowledge, provide training to their staff, and educate their patients. Although there are numerous educational resources available to dermatologists, an informal survey of 30 dermatology faculty members revealed a practice gap in awareness and utilization of these expanding electronic resources.¹¹

To better understand the needs of the specialty as a whole, we chose to focus on one aspect of dermatology education: resident training. The goal of our study was to survey dermatology residents and faculty to gain a better understanding of how they currently provide education and what online resources and social networking sites they currently use or would be willing to use. The study included 3 central hypotheses: First, residents would be less satisfied with their current curriculum and residents would report greater contributions to the curriculum relative to faculty. Second, both residents and faculty of smaller programs would be more interested in collaborative educational resources relative to larger programs. Lastly, residents would be more willing than faculty to participate in social networking for educational purposes.

Methods

This study was granted institutional review board exemption. Two surveys were developed by the authors to assess the current structure and satisfaction of dermatology residency curriculum and the willingness to participate in social networking to use and share educational materials. The surveys were evaluated for relevance by the survey evaluation team of the Association of Professors of Dermatology (APD). The instrument was not pilot tested.

The surveys were electronically distributed using an online service to dermatology faculty via the APD listserve, which comprised the entirety of the APD membership in 2014. The resident survey was distributed to the dermatology residents via the American Society for Dermatologic Surgery listserve, which included all residents in training (2013-2014 academic year). Second and third invitations to complete the surveys were distributed 3 and 5 weeks later, respectively.

Resident and faculty responses were compared. Additionally, responses were stratified for large (>9 residents) and small programs (≤9 residents) for comparison. Descriptive statistics including means and medians for continuous variables and frequency tables for categorical variables were generated using research and spreadsheet software.

Results

There were 137 survey respondents; 52 of 426 (12.2%) dermatology faculty and 85 of 1539 (5.5%) dermatology residents responded to the survey. Small programs accounted for 24% of total survey responses and 76% were from large programs.

Current Curriculum

The majority of dermatology faculty (44%) and residents (35%) identified 1 to 2 faculty members as contributing to the creation and organization of their respective curricula; however, a notable percentage of residents (9%) reported that no faculty contributed to the organization of the curriculum. Residents noted that senior residents carry twice the responsibility for structuring the curriculum compared to faculty (61% vs 32% of the workload), but faculty described an even split between senior residents and faculty (47% vs 49% of the workload). Faculty believed their residents spend a similar amount of time in resident- and faculty-led instruction (38% vs 35% of their time); however, the majority of residents reported spending too little time in faculty-led instruction (53%). When residents ranked their preference for learning modes, faculty-led and self-study learning were ranked first and second by 48% and 45% of residents, respectively. Resident-led instruction was ranked last by 66% of residents. Likewise, a majority of residents (53%) described their amount of time in faculty-led instruction as too little.

When asked what subjects in dermatology were lacking at their programs, residents reported clinical trials (47%), skin of color (46%), cosmetic dermatology (34%), and aggressive skin cancer/multidisciplinary tumor board (32%). Although 11% of residents reported lacking inpatient dermatology in their curriculum, 0% of faculty reported the same. A notable percentage of faculty reported nothing was lacking compared to residents (25% vs 7%). Despite these different views between residents and faculty on their contributions to and structure of their curriculums, both faculty and residents claimed overall satisfaction (satisfied or very satisfied) with their program’s ability to optimally cover the field of dermatology in 3 years (100% and 91%, respectively).

Large Versus Small Residency Programs

When stratifying the resident responses for small versus large programs, both program sizes reported more time in resident-led instruction than faculty-led instruction. Likewise, residents in both program sizes equally preferred self-study or faculty-led instruction to resident-led instruction. Residents at small programs more often reported lacking instruction in rheumatology, immunobullous diseases, and basic science/skin biology compared to large-program residents. Compared to large-program faculty, small-program faculty reported lacking instruction in cosmetic dermatology.

Faculty at small programs reported spending too little time preparing for their faculty-led instruction compared to faculty at large programs (44% vs 12%). All (100%) of the faculty at small programs were likely to seek out study materials shared by top educators, while 77% of faculty at large programs were likely to do the same. When asked if faculty would translate what their program does well into an electronic format for sharing, 30% of large-program faculty were likely to do so compared to 11% of small-program faculty (Figure 1).

Use of Online Educational Materials and Interest in Collaboration

A majority of faculty and residents stated that they use online educational materials as supplements to traditional classroom lecture and print materials (81% vs 86%); however, almost twice as many residents stated that online educational materials were essential to their current study routines compared to faculty (39% vs 21%).

The majority of faculty (92%) and residents (84%) were either interested or very interested in a collaborative online curriculum. Both residents (85%) and faculty (81%) stated they would be likely to seek out online educational materials shared by top educators. Although both residents and faculty reported many aspects of their curriculums they thought could be beneficial to other dermatology programs (Table 1), only 27% of faculty and 19% of residents were likely to translate those strengths into a shareable electronic format. Several reasons were reported for not contributing to an online curriculum, with lack of time being the most common reason (Table 2).

Eighty percent of residents and 88% of faculty reported they were either interested or very interested in being more connected/interactive with their dermatology peers nationally (Figure 2). Likewise, 94% of residents and 87% of faculty agreed that the dermatology community could benefit from a social networking site for educational collaboration. Four times as many residents versus faculty currently use social networking sites (eg, Facebook, LinkedIn, Google Groups) as a primary mode of communication with distant professional peers. The majority of residents (52%) reported they would be likely to participate in a professional social networking site, while the majority of faculty (50%) stated they were neutral on their likelihood of participating. Both residents and faculty reported lack of time as a common reason for being unlikely to utilize a professional social networking site. Other barriers to participation are listed in Table 3.

Comment

This study showed how dermatology faculty and residents currently provide training and what online resources and social networking sites they currently use or would be willing to use. The generalizability of the conclusions is limited by the low response rate for the surveys. The results demonstrated the different views between faculty and residents and between large and small residency programs on various topics. This microcosm of dermatology training can likely be applied to other training scenarios in dermatology, including patient education; training of nurses, physician extenders, and office staff; continuing medical education for physicians; and peer-to-peer collaboration.

Hypothesis 1: Partially Proven

We hypothesized that residents would report less satisfaction with their current curriculum and would report greater resident contributions to the curriculum relative to faculty. Overall, residents and faculty reported satisfaction with their curriculums to provide up-to-date information and breadth in the field of dermatology. Despite their overall satisfaction, more residents reported lacking instruction in several dermatology subtopics compared to faculty. Additionally, residents believed they spend twice as much time structuring their curriculum compared to faculty, with some residents reporting no faculty involvement. Although residents preferred faculty-led instruction, a majority of residents reported they do not have enough faculty-led didactics. The preference for faculty-led training is likely due to the expertise of faculty compared to residents.

Hypothesis 2: Partially Proven

We also hypothesized that both residents and faculty of smaller programs would be more interested in collaborative educational resources relative to larger programs. Although there was no difference in interest between residents at small versus large programs, there was a difference between faculty at small versus large programs. Small-program faculty were more interested in using shared materials than larger programs, while large-program faculty were more likely to share their educational materials. Small-program faculty reported spending too little time preparing their lectures, which is possibly due to a lack of time for preparation. Additionally, residents and faculty at smaller programs report their curriculum was lacking specific dermatology topics compared to large programs. These disparities between program sizes indicate a need for a social networking site for training collaboration in dermatology. Large programs have the ability to share what they do well, which small programs are eager to utilize.

Hypothesis 3: Not Proven

We hypothesized that residents would be more willing than faculty to participate in social networking for educational purposes. The majority of faculty and residents were interested in participating in a collaborative online curriculum and using the shared materials from top educators; however, even though such large majorities favored collaboration and sharing, only 27% of faculty and 19% of residents were likely to translate their own materials into a shareable format. Although lack of time was the most common reason for not sharing materials, electronic methods may have the potential to ultimately save time and remove the burden of content creation. The time it would take to translate selected personal training materials into a shareable form would be made up for by the time saved using another educators’ materials. Updating and customizing shared online educational materials can be much quicker and easier than educators creating materials on their own. Dermatologists would be more efficient facilitators of training via high-quality shared materials while decreasing the time burden associated with resident education.⁵ Another concern for not sharing or participating in a social networking site was skepticism of information security on such a network. The poor organization and information overload of online resources can compound the already existing time constraints on dermatologists, which may limit their ability to utilize such valuable resources. In addition, quality of online resources is not always guaranteed, and determining the sources that are high quality is sometimes a difficult task.⁶ For online materials to remain useful, there should be a peer-review process to evaluate quality and assess satisfaction.⁵

Solution: Create a Dermatology Task Force

A dermatology task force could facilitate the resolution of these challenges of online materials. In addition, a task force could cover the administrative support needed to ensure security and provide maintenance on social networks.

The main limitation to implementing a social network is the presence of the administrative infrastructure to jumpstart its creation. A task force incorporating the essential stakeholders in dermatology training is the first step. With inclusive representation from all of the smaller professional dermatology societies, the American Academy of Dermatology is optimally positioned to create this task force. With existing information technologies, a task force could address the concerns revealed in our survey as well as any future concerns that may arise.

The goal is a single social network for dermatologists that has the capability of improving communication and collaboration between professional peers regardless of their practice setting. Such a network is ideal for the practicing dermatologist for the purposes of staff training, patient education, and obtaining continuing medical education credit. Additionally, peer group collaboration would facilitate the understanding and completion of the evolving requirements for Maintenance of Certification from the American Board of Dermatology. The availability of quality shared materials would save time and increase efficiency of an entire dermatology practice. Materials that aid in patient education would allow office staff to dedicate their time to other tasks, thereby increasing productivity. Shared training materials would decrease the burden of staff education, providing more time for advanced hands-on training. This method of collaborative effort is capable of advancing the field of dermatology as a whole. It can overcome geographical and institutional barriers to connect dermatologists with similar interests worldwide; disseminate advances in diagnosis and treatment; and improve the quality of dermatology training of dermatologists, staff, and patients.

To the Editor:
Collagenous and elastotic marginal plaques of the hands (CEMPHs) has several names including degenerative collagenous plaques of the hands, keratoelastoidosis marginalis, and digital papular calcific elastosis. This rare disorder is an acquired, slowly progressive, asymptomatic, dermal connective tissue abnormality that is underrecognized and underdiagnosed. Clinical presentation includes hyperkeratotic translucent papules arranged linearly on the radial aspect of the hands.

A 74-year-old woman described having "rough hands" of more than 20 years' duration. She presented with 4-cm wide longitudinal, erythematous, firm, depressed plaques along the lateral edge of the second finger and extending to the medial thumb in both hands (Figure 1). She had attempted multiple treatments by her primary care physician, including topical and oral medications unknown to the patient and light therapy, all without benefit over a period of several years. We have attempted salicylic acid 40%, clobetasol cream 0.05%, and emollient creams containing α-hydroxy acid. At best the condition fluctuated between a subtle raised scale at the edge to smooth and occasionally more red-pink, seemingly unrelated to any treatments.

The patient did not have plaques elsewhere on the body, and notably, the feet were clear. She did not have a history of repeated trauma to the hands and did not engage in manual labor. She denied excessive sun exposure, though she had Fitzpatrick skin type III and a history of multiple precancers and nonmelanoma skin cancers 7 years prior to presentation.

Histology of CEMPH reveals a hyperkeratotic epidermis with an avascular and acellular replacement of the superficial reticular dermis by haphazardly arranged, thickened collagen fibers (Figure 2A-2C). Collagen fibers were oriented perpendicularly to the epidermal surface. Intervening amorphous basophilic elastotic masses were present in the upper dermis with occasional calcification and degenerative elastic fibers (Figure 2D).

Collagenous and elastotic marginal plaques of the hands is a chronic, asymptomatic, sclerotic skin disorder described in a 1960 case series of 5 patients reported by Burks et al.¹ Although it has many names, the most common is CEMPH. Collagenous and elastotic marginal plaques of the hands most often presents in white men aged 50 to 60 years.² Patients typically are asymptomatic with plaques limited to the junction of the palmar and dorsal surfaces of the hands with only minimal intermittent stiffness around the flexor creases. Lesions begin as discrete yellow papules that coalesce to form hyperkeratotic linear plaques with occasional telangiectasia.³

The etiology of CEMPH is attributed to collagen and elastin degeneration by chronic actinic damage, pressure, or trauma.^4,5 The 3 stages of degeneration include an initial linear padded stage, an intermediate padded plaque stage, and an advanced padded hyperkeratotic plaque stage.⁴ Vascular compromise is seen from the enlarged and fused thickened collagen and elastic fibers that in turn lead to ischemic changes, hyperkeratosis with epidermal atrophy, and papillary dermis telangiectasia. Absence or weak expression of keratins 14 and 10 and strong expression of keratin 16 have been reported in the epidermis of CEMPH patients.⁴

Collagenous and elastotic marginal plaques of the hands do not have a specific treatment, as it is a benign, slowly progressive condition. Several treatments such as laser therapy, high-potency topical corticosteroids, topical tazarotene and tretinoin, oral isotretinoin, and cryotherapy have been tried with little long-term success.⁴ Moisturizing may help reduce fissuring, and patients are advised to avoid the sun and repeated trauma to the hands.

The differential diagnosis of CEMPH is summarized in the Table. Two genodermatoses—acrokeratoelastoidosis of Costa and focal acral hyperkeratosis—clinically resemble CEMPH. Acrokeratoelastoidosis of Costa is an autosomal-dominant condition that occurs without trauma in children and young adults. Histopathology shows orthokeratotic hyperkeratosis due to an overproduction of filaggrin in the granular layer of the epidermis. The reticular dermis shows basophilic, thick, curled and fragmented elastic fibers with dilated capillaries that can be seen with Weigert elastic, Verhoeff-van Gieson, or orcein stains. Focal acral hyperkeratosis occurs on the hands and feet, predominantly in black patients. On histology, the epidermis shows a characteristic orthohyperkeratosis, moderate acanthosis, and slight hypergranulosis with no dermal involvment.⁶

Chronic hyperkeratotic eczematous dermatitis is another common entity in the differential characterized by hyperkeratotic plaques that scale and fissure. Biopsy demonstrates a spongiotic acanthotic epidermis.^7,8

Psoriasis of the hands, specifically hyperkeratotic palmoplantar psoriasis, is associated with manual labor, similar to CEMPH. Histology shows epidermal hyperplasia; regular acanthosis; loss of the granular skin layer with prominent dermal capillaries; and a mixed dermal infiltrate of lymphocytes, macrophages, and neutrophils.⁹ Hyperkeratotic palmoplantar lichen planus presents with pruritic papules in the third and fifth decades of life. Histologically, hyperkeratosis, acanthosis, and wedge-shaped hypergranulosis with a lichenoid lymphocytic infiltration at the dermoepidermal junction is seen.¹⁰

Palmoplantar keratodermas due to inflammatory reactive dermatoses include callosities that develop in response to repeated trauma or friction on the skin. On histology, there is prominent hyperkeratosis and acanthosis with moderate papillomatosis.¹¹ Drug-related palmoplantar keratodermas such as those from arsenic exposure can lead to multiple, irregular, verrucous, keratotic, and pigmented lesions on the palms and soles. Histologically, atypical keratinocytes are seen in the epidermis with thick hyperkeratosis and vacuolated cells without solar elastosis.¹²

In conclusion, CEMPH is an underdiagnosed and underrecognized condition characterized by asymptomatic hyperkeratotic linear plaques along the medial aspect of the thumb and radial aspect of the index finger. It is important to keep CEMPH in mind when dealing with occupational cases of repeated long-term trauma or pressure to the hands as well as excessive sun exposure. It also is imperative to separate it from other diseases and avoid misdiagnosing this degenerative collagenous and elastotic disease as a malignant lesion. 

To the Editor:
Collagenous and elastotic marginal plaques of the hands (CEMPHs) has several names including degenerative collagenous plaques of the hands, keratoelastoidosis marginalis, and digital papular calcific elastosis. This rare disorder is an acquired, slowly progressive, asymptomatic, dermal connective tissue abnormality that is underrecognized and underdiagnosed. Clinical presentation includes hyperkeratotic translucent papules arranged linearly on the radial aspect of the hands.

A 74-year-old woman described having "rough hands" of more than 20 years' duration. She presented with 4-cm wide longitudinal, erythematous, firm, depressed plaques along the lateral edge of the second finger and extending to the medial thumb in both hands (Figure 1). She had attempted multiple treatments by her primary care physician, including topical and oral medications unknown to the patient and light therapy, all without benefit over a period of several years. We have attempted salicylic acid 40%, clobetasol cream 0.05%, and emollient creams containing α-hydroxy acid. At best the condition fluctuated between a subtle raised scale at the edge to smooth and occasionally more red-pink, seemingly unrelated to any treatments.

The patient did not have plaques elsewhere on the body, and notably, the feet were clear. She did not have a history of repeated trauma to the hands and did not engage in manual labor. She denied excessive sun exposure, though she had Fitzpatrick skin type III and a history of multiple precancers and nonmelanoma skin cancers 7 years prior to presentation.

Histology of CEMPH reveals a hyperkeratotic epidermis with an avascular and acellular replacement of the superficial reticular dermis by haphazardly arranged, thickened collagen fibers (Figure 2A-2C). Collagen fibers were oriented perpendicularly to the epidermal surface. Intervening amorphous basophilic elastotic masses were present in the upper dermis with occasional calcification and degenerative elastic fibers (Figure 2D).

Collagenous and elastotic marginal plaques of the hands is a chronic, asymptomatic, sclerotic skin disorder described in a 1960 case series of 5 patients reported by Burks et al.¹ Although it has many names, the most common is CEMPH. Collagenous and elastotic marginal plaques of the hands most often presents in white men aged 50 to 60 years.² Patients typically are asymptomatic with plaques limited to the junction of the palmar and dorsal surfaces of the hands with only minimal intermittent stiffness around the flexor creases. Lesions begin as discrete yellow papules that coalesce to form hyperkeratotic linear plaques with occasional telangiectasia.³

The etiology of CEMPH is attributed to collagen and elastin degeneration by chronic actinic damage, pressure, or trauma.^4,5 The 3 stages of degeneration include an initial linear padded stage, an intermediate padded plaque stage, and an advanced padded hyperkeratotic plaque stage.⁴ Vascular compromise is seen from the enlarged and fused thickened collagen and elastic fibers that in turn lead to ischemic changes, hyperkeratosis with epidermal atrophy, and papillary dermis telangiectasia. Absence or weak expression of keratins 14 and 10 and strong expression of keratin 16 have been reported in the epidermis of CEMPH patients.⁴

Collagenous and elastotic marginal plaques of the hands do not have a specific treatment, as it is a benign, slowly progressive condition. Several treatments such as laser therapy, high-potency topical corticosteroids, topical tazarotene and tretinoin, oral isotretinoin, and cryotherapy have been tried with little long-term success.⁴ Moisturizing may help reduce fissuring, and patients are advised to avoid the sun and repeated trauma to the hands.

The differential diagnosis of CEMPH is summarized in the Table. Two genodermatoses—acrokeratoelastoidosis of Costa and focal acral hyperkeratosis—clinically resemble CEMPH. Acrokeratoelastoidosis of Costa is an autosomal-dominant condition that occurs without trauma in children and young adults. Histopathology shows orthokeratotic hyperkeratosis due to an overproduction of filaggrin in the granular layer of the epidermis. The reticular dermis shows basophilic, thick, curled and fragmented elastic fibers with dilated capillaries that can be seen with Weigert elastic, Verhoeff-van Gieson, or orcein stains. Focal acral hyperkeratosis occurs on the hands and feet, predominantly in black patients. On histology, the epidermis shows a characteristic orthohyperkeratosis, moderate acanthosis, and slight hypergranulosis with no dermal involvment.⁶

Chronic hyperkeratotic eczematous dermatitis is another common entity in the differential characterized by hyperkeratotic plaques that scale and fissure. Biopsy demonstrates a spongiotic acanthotic epidermis.^7,8

Psoriasis of the hands, specifically hyperkeratotic palmoplantar psoriasis, is associated with manual labor, similar to CEMPH. Histology shows epidermal hyperplasia; regular acanthosis; loss of the granular skin layer with prominent dermal capillaries; and a mixed dermal infiltrate of lymphocytes, macrophages, and neutrophils.⁹ Hyperkeratotic palmoplantar lichen planus presents with pruritic papules in the third and fifth decades of life. Histologically, hyperkeratosis, acanthosis, and wedge-shaped hypergranulosis with a lichenoid lymphocytic infiltration at the dermoepidermal junction is seen.¹⁰

Palmoplantar keratodermas due to inflammatory reactive dermatoses include callosities that develop in response to repeated trauma or friction on the skin. On histology, there is prominent hyperkeratosis and acanthosis with moderate papillomatosis.¹¹ Drug-related palmoplantar keratodermas such as those from arsenic exposure can lead to multiple, irregular, verrucous, keratotic, and pigmented lesions on the palms and soles. Histologically, atypical keratinocytes are seen in the epidermis with thick hyperkeratosis and vacuolated cells without solar elastosis.¹²

In conclusion, CEMPH is an underdiagnosed and underrecognized condition characterized by asymptomatic hyperkeratotic linear plaques along the medial aspect of the thumb and radial aspect of the index finger. It is important to keep CEMPH in mind when dealing with occupational cases of repeated long-term trauma or pressure to the hands as well as excessive sun exposure. It also is imperative to separate it from other diseases and avoid misdiagnosing this degenerative collagenous and elastotic disease as a malignant lesion. 

To the Editor:
Collagenous and elastotic marginal plaques of the hands (CEMPHs) has several names including degenerative collagenous plaques of the hands, keratoelastoidosis marginalis, and digital papular calcific elastosis. This rare disorder is an acquired, slowly progressive, asymptomatic, dermal connective tissue abnormality that is underrecognized and underdiagnosed. Clinical presentation includes hyperkeratotic translucent papules arranged linearly on the radial aspect of the hands.

A 74-year-old woman described having "rough hands" of more than 20 years' duration. She presented with 4-cm wide longitudinal, erythematous, firm, depressed plaques along the lateral edge of the second finger and extending to the medial thumb in both hands (Figure 1). She had attempted multiple treatments by her primary care physician, including topical and oral medications unknown to the patient and light therapy, all without benefit over a period of several years. We have attempted salicylic acid 40%, clobetasol cream 0.05%, and emollient creams containing α-hydroxy acid. At best the condition fluctuated between a subtle raised scale at the edge to smooth and occasionally more red-pink, seemingly unrelated to any treatments.

The patient did not have plaques elsewhere on the body, and notably, the feet were clear. She did not have a history of repeated trauma to the hands and did not engage in manual labor. She denied excessive sun exposure, though she had Fitzpatrick skin type III and a history of multiple precancers and nonmelanoma skin cancers 7 years prior to presentation.

Histology of CEMPH reveals a hyperkeratotic epidermis with an avascular and acellular replacement of the superficial reticular dermis by haphazardly arranged, thickened collagen fibers (Figure 2A-2C). Collagen fibers were oriented perpendicularly to the epidermal surface. Intervening amorphous basophilic elastotic masses were present in the upper dermis with occasional calcification and degenerative elastic fibers (Figure 2D).

Collagenous and elastotic marginal plaques of the hands is a chronic, asymptomatic, sclerotic skin disorder described in a 1960 case series of 5 patients reported by Burks et al.¹ Although it has many names, the most common is CEMPH. Collagenous and elastotic marginal plaques of the hands most often presents in white men aged 50 to 60 years.² Patients typically are asymptomatic with plaques limited to the junction of the palmar and dorsal surfaces of the hands with only minimal intermittent stiffness around the flexor creases. Lesions begin as discrete yellow papules that coalesce to form hyperkeratotic linear plaques with occasional telangiectasia.³

The etiology of CEMPH is attributed to collagen and elastin degeneration by chronic actinic damage, pressure, or trauma.^4,5 The 3 stages of degeneration include an initial linear padded stage, an intermediate padded plaque stage, and an advanced padded hyperkeratotic plaque stage.⁴ Vascular compromise is seen from the enlarged and fused thickened collagen and elastic fibers that in turn lead to ischemic changes, hyperkeratosis with epidermal atrophy, and papillary dermis telangiectasia. Absence or weak expression of keratins 14 and 10 and strong expression of keratin 16 have been reported in the epidermis of CEMPH patients.⁴

Collagenous and elastotic marginal plaques of the hands do not have a specific treatment, as it is a benign, slowly progressive condition. Several treatments such as laser therapy, high-potency topical corticosteroids, topical tazarotene and tretinoin, oral isotretinoin, and cryotherapy have been tried with little long-term success.⁴ Moisturizing may help reduce fissuring, and patients are advised to avoid the sun and repeated trauma to the hands.

The differential diagnosis of CEMPH is summarized in the Table. Two genodermatoses—acrokeratoelastoidosis of Costa and focal acral hyperkeratosis—clinically resemble CEMPH. Acrokeratoelastoidosis of Costa is an autosomal-dominant condition that occurs without trauma in children and young adults. Histopathology shows orthokeratotic hyperkeratosis due to an overproduction of filaggrin in the granular layer of the epidermis. The reticular dermis shows basophilic, thick, curled and fragmented elastic fibers with dilated capillaries that can be seen with Weigert elastic, Verhoeff-van Gieson, or orcein stains. Focal acral hyperkeratosis occurs on the hands and feet, predominantly in black patients. On histology, the epidermis shows a characteristic orthohyperkeratosis, moderate acanthosis, and slight hypergranulosis with no dermal involvment.⁶

Chronic hyperkeratotic eczematous dermatitis is another common entity in the differential characterized by hyperkeratotic plaques that scale and fissure. Biopsy demonstrates a spongiotic acanthotic epidermis.^7,8

Psoriasis of the hands, specifically hyperkeratotic palmoplantar psoriasis, is associated with manual labor, similar to CEMPH. Histology shows epidermal hyperplasia; regular acanthosis; loss of the granular skin layer with prominent dermal capillaries; and a mixed dermal infiltrate of lymphocytes, macrophages, and neutrophils.⁹ Hyperkeratotic palmoplantar lichen planus presents with pruritic papules in the third and fifth decades of life. Histologically, hyperkeratosis, acanthosis, and wedge-shaped hypergranulosis with a lichenoid lymphocytic infiltration at the dermoepidermal junction is seen.¹⁰

Palmoplantar keratodermas due to inflammatory reactive dermatoses include callosities that develop in response to repeated trauma or friction on the skin. On histology, there is prominent hyperkeratosis and acanthosis with moderate papillomatosis.¹¹ Drug-related palmoplantar keratodermas such as those from arsenic exposure can lead to multiple, irregular, verrucous, keratotic, and pigmented lesions on the palms and soles. Histologically, atypical keratinocytes are seen in the epidermis with thick hyperkeratosis and vacuolated cells without solar elastosis.¹²

In conclusion, CEMPH is an underdiagnosed and underrecognized condition characterized by asymptomatic hyperkeratotic linear plaques along the medial aspect of the thumb and radial aspect of the index finger. It is important to keep CEMPH in mind when dealing with occupational cases of repeated long-term trauma or pressure to the hands as well as excessive sun exposure. It also is imperative to separate it from other diseases and avoid misdiagnosing this degenerative collagenous and elastotic disease as a malignant lesion. 

Ota nevus is a dermal melanocytosis that is typically characterized by blue, gray, or brown pigmented patches in the periorbital region.¹ The condition has a prevalence of 0.04% in a Philadelphia study of 6915 patients and is most notable in patients with skin of color, affecting up to 0.6% of Asians,² 0.038% of white individuals, and 0.014% of black individuals.^3,4 The appearance of an Ota nevus often imparts a negative psychosocial impact on the patient, prompting requests for treatment and/or removal.⁵Laser treatment of Ota nevi must be carefully implemented, especially in Fitzpatrick skin types IV through VI. Although 532- and 755-nm Q-switched nanosecond lasers have been used to treat Ota nevi,^5,6 typically only moderate improvement is seen; further treatment at higher fluences will only increase the risk for dyspigmentation and scarring.⁶

We report a case of successful treatment of an Ota nevus following 2 treatment sessions with the 532-nm solid-state picosecond laser, which is a novel application in patients with skin of color (Fitzpatrick skin types IV-VI). The Q-switched nanosecond laser has been shown to be moderately effective at treating Ota nevi.⁶ 

Case Report

An 18-year-old woman with Fitzpatrick skin type IV presented for cosmetic removal of an 8×5-cm dark brown-blue patch on the right temple and malar and buccal cheek present since birth that had failed to respond to an unknown laser treatment that was administered outside of the United States (Figure, A). To ascertain the diagnosis, a biopsy was performed, showing histology consistent with Ota nevus. Initially, the 755-nm Q-switched nanosecond laser was recommended for treatment. Over the course of 7 months (1 treatment session per month [Table]), the patient saw improvement but not to the desired extent. The patient then underwent 2 treatments at 4-week intervals with the 1064-nm solid-state picosecond and nanosecond lasers; however, no improvement was seen following these 2 sessions (Table).

The next month the patient received treatment with a novel 532-nm solid-state picosecond laser using the following parameters: fluence, 0.5 J/cm²; spot size, 6 mm; repetition rate, 1 Hz; pulse duration, 750 picoseconds; 339 pulses. The end point was whitening. A remarkable clinical response was demonstrated 6 weeks later (Figure, B). A second treatment with the 532-nm solid-state picosecond laser was then performed at 14 months. On a return visit 2 months after the second treatment, the patient showed dramatic improvement, almost to the degree of complete resolution (Figure, C). 

Comment

Pigmentation disorders are more common in patients with skin of color, and those affected may experience psychological effects secondary to these dermatoses, prompting requests for treatment and/or removal.⁷ Although the 532- and 755-nm Q-switched nanosecond lasers have been used to treat Ota nevi,³ the challenge remains for patients with skin of color, as these lasers work through photothermolysis, which generates heat and may cause thermal damage by targeting melanin. Because more melanin is present in skin of color patients, the threshold for too much heat is lower and these patients are at a higher risk for adverse events such as scarring and hyperpigmentation.^6,8

By delivering energy in shorter pulses, the novel 532-nm solid-state picosecond laser shows greater fragmentation of melanosomes into melanin particles that are eventually phagocytosed.⁸ In our patient, dramatic improvement was noted after only 2 treatments, as evidenced by other picosecond treatments on Ota nevi,^6,8 suggesting that fewer treatments are necessary when using the 532-nm solid-state picosecond laser for Ota nevi.

Although the 532-nm solid-state picosecond laser was cleared by the US Food and Drug Administration for tattoo removal, this laser shows potential use in other pigmentary disorders, particularly in patients with skin of color, as demonstrated in our case. With continued understanding through further studies, this picosecond laser with a shorter pulse duration may prove to be a safer and more effective alternative to the Q-switched nanosecond laser.

Conclusion

As shown in our case, the 532-nm solid-state picosecond laser appears to be a safe and effective modality for treating Ota nevi. This case demonstrates the potential utility of this laser in patients desiring more complete clearing, as it removes pigment more rapidly with lower risk for serious adverse effects. The 9th Cosmetic Surgery Forum will be held November 29-December 2, 2017, in Las Vegas, Nevada. Get more information at www.cosmeticsurgeryforum.com.

Ota nevus is a dermal melanocytosis that is typically characterized by blue, gray, or brown pigmented patches in the periorbital region.¹ The condition has a prevalence of 0.04% in a Philadelphia study of 6915 patients and is most notable in patients with skin of color, affecting up to 0.6% of Asians,² 0.038% of white individuals, and 0.014% of black individuals.^3,4 The appearance of an Ota nevus often imparts a negative psychosocial impact on the patient, prompting requests for treatment and/or removal.⁵Laser treatment of Ota nevi must be carefully implemented, especially in Fitzpatrick skin types IV through VI. Although 532- and 755-nm Q-switched nanosecond lasers have been used to treat Ota nevi,^5,6 typically only moderate improvement is seen; further treatment at higher fluences will only increase the risk for dyspigmentation and scarring.⁶

We report a case of successful treatment of an Ota nevus following 2 treatment sessions with the 532-nm solid-state picosecond laser, which is a novel application in patients with skin of color (Fitzpatrick skin types IV-VI). The Q-switched nanosecond laser has been shown to be moderately effective at treating Ota nevi.⁶ 

Case Report

An 18-year-old woman with Fitzpatrick skin type IV presented for cosmetic removal of an 8×5-cm dark brown-blue patch on the right temple and malar and buccal cheek present since birth that had failed to respond to an unknown laser treatment that was administered outside of the United States (Figure, A). To ascertain the diagnosis, a biopsy was performed, showing histology consistent with Ota nevus. Initially, the 755-nm Q-switched nanosecond laser was recommended for treatment. Over the course of 7 months (1 treatment session per month [Table]), the patient saw improvement but not to the desired extent. The patient then underwent 2 treatments at 4-week intervals with the 1064-nm solid-state picosecond and nanosecond lasers; however, no improvement was seen following these 2 sessions (Table).

The next month the patient received treatment with a novel 532-nm solid-state picosecond laser using the following parameters: fluence, 0.5 J/cm²; spot size, 6 mm; repetition rate, 1 Hz; pulse duration, 750 picoseconds; 339 pulses. The end point was whitening. A remarkable clinical response was demonstrated 6 weeks later (Figure, B). A second treatment with the 532-nm solid-state picosecond laser was then performed at 14 months. On a return visit 2 months after the second treatment, the patient showed dramatic improvement, almost to the degree of complete resolution (Figure, C). 

Comment

Pigmentation disorders are more common in patients with skin of color, and those affected may experience psychological effects secondary to these dermatoses, prompting requests for treatment and/or removal.⁷ Although the 532- and 755-nm Q-switched nanosecond lasers have been used to treat Ota nevi,³ the challenge remains for patients with skin of color, as these lasers work through photothermolysis, which generates heat and may cause thermal damage by targeting melanin. Because more melanin is present in skin of color patients, the threshold for too much heat is lower and these patients are at a higher risk for adverse events such as scarring and hyperpigmentation.^6,8

By delivering energy in shorter pulses, the novel 532-nm solid-state picosecond laser shows greater fragmentation of melanosomes into melanin particles that are eventually phagocytosed.⁸ In our patient, dramatic improvement was noted after only 2 treatments, as evidenced by other picosecond treatments on Ota nevi,^6,8 suggesting that fewer treatments are necessary when using the 532-nm solid-state picosecond laser for Ota nevi.

Although the 532-nm solid-state picosecond laser was cleared by the US Food and Drug Administration for tattoo removal, this laser shows potential use in other pigmentary disorders, particularly in patients with skin of color, as demonstrated in our case. With continued understanding through further studies, this picosecond laser with a shorter pulse duration may prove to be a safer and more effective alternative to the Q-switched nanosecond laser.

Conclusion

As shown in our case, the 532-nm solid-state picosecond laser appears to be a safe and effective modality for treating Ota nevi. This case demonstrates the potential utility of this laser in patients desiring more complete clearing, as it removes pigment more rapidly with lower risk for serious adverse effects. The 9th Cosmetic Surgery Forum will be held November 29-December 2, 2017, in Las Vegas, Nevada. Get more information at www.cosmeticsurgeryforum.com.

Ota nevus is a dermal melanocytosis that is typically characterized by blue, gray, or brown pigmented patches in the periorbital region.¹ The condition has a prevalence of 0.04% in a Philadelphia study of 6915 patients and is most notable in patients with skin of color, affecting up to 0.6% of Asians,² 0.038% of white individuals, and 0.014% of black individuals.^3,4 The appearance of an Ota nevus often imparts a negative psychosocial impact on the patient, prompting requests for treatment and/or removal.⁵Laser treatment of Ota nevi must be carefully implemented, especially in Fitzpatrick skin types IV through VI. Although 532- and 755-nm Q-switched nanosecond lasers have been used to treat Ota nevi,^5,6 typically only moderate improvement is seen; further treatment at higher fluences will only increase the risk for dyspigmentation and scarring.⁶

We report a case of successful treatment of an Ota nevus following 2 treatment sessions with the 532-nm solid-state picosecond laser, which is a novel application in patients with skin of color (Fitzpatrick skin types IV-VI). The Q-switched nanosecond laser has been shown to be moderately effective at treating Ota nevi.⁶ 

Case Report

An 18-year-old woman with Fitzpatrick skin type IV presented for cosmetic removal of an 8×5-cm dark brown-blue patch on the right temple and malar and buccal cheek present since birth that had failed to respond to an unknown laser treatment that was administered outside of the United States (Figure, A). To ascertain the diagnosis, a biopsy was performed, showing histology consistent with Ota nevus. Initially, the 755-nm Q-switched nanosecond laser was recommended for treatment. Over the course of 7 months (1 treatment session per month [Table]), the patient saw improvement but not to the desired extent. The patient then underwent 2 treatments at 4-week intervals with the 1064-nm solid-state picosecond and nanosecond lasers; however, no improvement was seen following these 2 sessions (Table).

The next month the patient received treatment with a novel 532-nm solid-state picosecond laser using the following parameters: fluence, 0.5 J/cm²; spot size, 6 mm; repetition rate, 1 Hz; pulse duration, 750 picoseconds; 339 pulses. The end point was whitening. A remarkable clinical response was demonstrated 6 weeks later (Figure, B). A second treatment with the 532-nm solid-state picosecond laser was then performed at 14 months. On a return visit 2 months after the second treatment, the patient showed dramatic improvement, almost to the degree of complete resolution (Figure, C). 

Comment

Pigmentation disorders are more common in patients with skin of color, and those affected may experience psychological effects secondary to these dermatoses, prompting requests for treatment and/or removal.⁷ Although the 532- and 755-nm Q-switched nanosecond lasers have been used to treat Ota nevi,³ the challenge remains for patients with skin of color, as these lasers work through photothermolysis, which generates heat and may cause thermal damage by targeting melanin. Because more melanin is present in skin of color patients, the threshold for too much heat is lower and these patients are at a higher risk for adverse events such as scarring and hyperpigmentation.^6,8

By delivering energy in shorter pulses, the novel 532-nm solid-state picosecond laser shows greater fragmentation of melanosomes into melanin particles that are eventually phagocytosed.⁸ In our patient, dramatic improvement was noted after only 2 treatments, as evidenced by other picosecond treatments on Ota nevi,^6,8 suggesting that fewer treatments are necessary when using the 532-nm solid-state picosecond laser for Ota nevi.

Although the 532-nm solid-state picosecond laser was cleared by the US Food and Drug Administration for tattoo removal, this laser shows potential use in other pigmentary disorders, particularly in patients with skin of color, as demonstrated in our case. With continued understanding through further studies, this picosecond laser with a shorter pulse duration may prove to be a safer and more effective alternative to the Q-switched nanosecond laser.

Conclusion

As shown in our case, the 532-nm solid-state picosecond laser appears to be a safe and effective modality for treating Ota nevi. This case demonstrates the potential utility of this laser in patients desiring more complete clearing, as it removes pigment more rapidly with lower risk for serious adverse effects. The 9th Cosmetic Surgery Forum will be held November 29-December 2, 2017, in Las Vegas, Nevada. Get more information at www.cosmeticsurgeryforum.com.

ORLANDO – A two-step regimen of high-potency topical antioxidants followed by a mineral-based sunscreen may help repair light-induced skin damage and protect against new damage in patients with melasma.

Recent studies suggest that the antioxidants tamp down inflammatory cytokines and damage of oxidative stress, Maria Ivonne Arellano-Mendoza, MD, said during a special focus session on Latino skin held at the annual meeting of the American Academy of Dermatology.

“Many compounds are being studied for this purpose,” said Dr. Arellano-Mendoza, head of the dermatology department of the General Hospital of Mexico, Mexico City. “One combination is vitamin C, vitamin E, ubiquinone, and grape-seed extract. This effectively prevented infrared-A radiation–induced matrix metalloproteinase-1 messenger RNA expression in human skin. This combination can be found now in some sunscreens and daily care products.”

Another effective combination seems to be a mixture of ferulic acid, tocopherol, and vitamin C, she said.

The two-step process of regularly using a topical product with antioxidants before applying a sunscreen is all there is for now, she added, because so far it’s been impossible to combine the agents in a single product.

“The challenge will be how to create a product that stays on the surface of the skin to protect it from light, while liberating the antioxidants to penetrate the skin,” she commented.

The antioxidants’ benefits, however, will be obliterated by the continued effects of some light wavelengths that aggravate melasma unless they are used in sequence with a light-scattering sunscreen.

Sunscreens are critical components of a melasma treatment regimen, Dr. Arellano-Mendoza said. “Sunscreens are a cornerstone of treatment. We clearly tell our patients that sunscreens have to be used every day, forever, and if they are not used properly, they will have no improvement.”

Patients with hyperpigmentation disorders are susceptible to longer wavelengths that aren’t covered by chemically derived sunscreens. Longer wavelengths, including infrared light and visible light, have been shown to increase expression of matrix metalloproteinase (MMP) -1 and -9, decrease expression of type 1 procollagen, and can induce macrophage infiltration. These wavelengths also increase reactive oxygen species and proinflammatory cytokines in vitro, Dr. Arellano-Mendoza noted.

Visible light can cause erythema, transient and long-lasting hyperpigmentation, thermal damage, free radical production, and premature photoaging. It also can stimulate the production of reactive oxygen species that can damage DNA.

Mineral-based, inorganic sunscreens, however – like those with titanium dioxide, zinc oxide, and iron oxide – scatter all wavelengths.

“These micronized forms of metal oxides not only scatter and reflect light, they also absorb ultraviolet radiation. The compounds aren’t new,” she said. In 1991, Dr. Elaine Kaye of Harvard University, Boston, and associates described (Arch Dermatol. 1991;127:351-5) opaque physical sunscreens that were useful blockers of visible light and found that transmittance of light can be lowered by adding iron oxide, Dr. Arellano-Mendoza pointed out.

The inorganic sunscreens have never been widely adopted because they are highly pigmented with white or, in the case of iron oxide, with red. “Not many people accepted [the iron-containing compounds] because of the redness, but now different shades are going to be hitting the market soon,” and the hope is that consumers will find them more appealing, she said.

This is good news, as the data emerging around iron oxide are intriguingly positive. A 2015 study showed that a sunscreen with iron oxides prevented melasma relapse during the summer months. Patients were randomized to the same ultraviolet filter topical sunscreen, but for one group, micronized iron oxide was added to it. After 6 months, the median melasma area severity index score was significantly better in the group using the iron oxide compound (J Am Acad Dermatol. 2015 Jan;72[1]:189-90.e1).

Support for the two-step regimen appeared in 2014, when a small study randomized 30 healthy volunteers to an SPF 30 sunscreen or the same sunscreen supplemented with an antioxidant cocktail of grape seed extract, vitamin E, ubiquinone, and vitamin C. The endpoint was MMP-1 upregulation after exposure to infrared-A light. Skin treated with the combination regimen showed significantly lower MMP-1 activation, leading the authors to conclude that the combination of topical antioxidants conferred protection against the irradiation (Photochem Photobiol. 2015 Jan-Feb;91[1]:248-50).

Those same authors published a companion article (Photodermatol Photoimmunol Photomed. 2014;30:167-74) suggesting that another antioxidant mixture (ferulic acid, tocopherol, and vitamin C) was similarly effective.

Using a combination of antioxidants is important, Dr. Arellano-Mendoza said, because different antioxidants work differently. Some (catalase, glutathione peroxidase, and superoxide dismutase) are enzymatic, catalyzing reactions that convert free radicals to oxygen and water. Others terminate free radicals by preventing the propagation of oxidative chain reactions (vitamins A and C, flavonoids, uric acid, bilirubin, albumin, and members of the thiol group). A third group consists of metal-binding proteins that sequester free iron or copper to prevent free radical production (ferritin, transferrin, lactoferrin, and ceruloplasmin).

“I think we can now consider antioxidants a part of the tools we use in treating some pigmentary disorders as melasma,” she said. “We need to choose the compounds carefully, and we definitely need more in vivo research, but the findings are very encouraging.”

Dr. Arellano-Mendoza had no relevant financial disclosures.

This article was updated 3/20/17.
 

[email protected]

On Twitter @Alz_Gal

ORLANDO – A two-step regimen of high-potency topical antioxidants followed by a mineral-based sunscreen may help repair light-induced skin damage and protect against new damage in patients with melasma.

Recent studies suggest that the antioxidants tamp down inflammatory cytokines and damage of oxidative stress, Maria Ivonne Arellano-Mendoza, MD, said during a special focus session on Latino skin held at the annual meeting of the American Academy of Dermatology.

“Many compounds are being studied for this purpose,” said Dr. Arellano-Mendoza, head of the dermatology department of the General Hospital of Mexico, Mexico City. “One combination is vitamin C, vitamin E, ubiquinone, and grape-seed extract. This effectively prevented infrared-A radiation–induced matrix metalloproteinase-1 messenger RNA expression in human skin. This combination can be found now in some sunscreens and daily care products.”

Another effective combination seems to be a mixture of ferulic acid, tocopherol, and vitamin C, she said.

The two-step process of regularly using a topical product with antioxidants before applying a sunscreen is all there is for now, she added, because so far it’s been impossible to combine the agents in a single product.

“The challenge will be how to create a product that stays on the surface of the skin to protect it from light, while liberating the antioxidants to penetrate the skin,” she commented.

The antioxidants’ benefits, however, will be obliterated by the continued effects of some light wavelengths that aggravate melasma unless they are used in sequence with a light-scattering sunscreen.

Sunscreens are critical components of a melasma treatment regimen, Dr. Arellano-Mendoza said. “Sunscreens are a cornerstone of treatment. We clearly tell our patients that sunscreens have to be used every day, forever, and if they are not used properly, they will have no improvement.”

Patients with hyperpigmentation disorders are susceptible to longer wavelengths that aren’t covered by chemically derived sunscreens. Longer wavelengths, including infrared light and visible light, have been shown to increase expression of matrix metalloproteinase (MMP) -1 and -9, decrease expression of type 1 procollagen, and can induce macrophage infiltration. These wavelengths also increase reactive oxygen species and proinflammatory cytokines in vitro, Dr. Arellano-Mendoza noted.

Visible light can cause erythema, transient and long-lasting hyperpigmentation, thermal damage, free radical production, and premature photoaging. It also can stimulate the production of reactive oxygen species that can damage DNA.

Mineral-based, inorganic sunscreens, however – like those with titanium dioxide, zinc oxide, and iron oxide – scatter all wavelengths.

“These micronized forms of metal oxides not only scatter and reflect light, they also absorb ultraviolet radiation. The compounds aren’t new,” she said. In 1991, Dr. Elaine Kaye of Harvard University, Boston, and associates described (Arch Dermatol. 1991;127:351-5) opaque physical sunscreens that were useful blockers of visible light and found that transmittance of light can be lowered by adding iron oxide, Dr. Arellano-Mendoza pointed out.

The inorganic sunscreens have never been widely adopted because they are highly pigmented with white or, in the case of iron oxide, with red. “Not many people accepted [the iron-containing compounds] because of the redness, but now different shades are going to be hitting the market soon,” and the hope is that consumers will find them more appealing, she said.

This is good news, as the data emerging around iron oxide are intriguingly positive. A 2015 study showed that a sunscreen with iron oxides prevented melasma relapse during the summer months. Patients were randomized to the same ultraviolet filter topical sunscreen, but for one group, micronized iron oxide was added to it. After 6 months, the median melasma area severity index score was significantly better in the group using the iron oxide compound (J Am Acad Dermatol. 2015 Jan;72[1]:189-90.e1).

Support for the two-step regimen appeared in 2014, when a small study randomized 30 healthy volunteers to an SPF 30 sunscreen or the same sunscreen supplemented with an antioxidant cocktail of grape seed extract, vitamin E, ubiquinone, and vitamin C. The endpoint was MMP-1 upregulation after exposure to infrared-A light. Skin treated with the combination regimen showed significantly lower MMP-1 activation, leading the authors to conclude that the combination of topical antioxidants conferred protection against the irradiation (Photochem Photobiol. 2015 Jan-Feb;91[1]:248-50).

Those same authors published a companion article (Photodermatol Photoimmunol Photomed. 2014;30:167-74) suggesting that another antioxidant mixture (ferulic acid, tocopherol, and vitamin C) was similarly effective.

Using a combination of antioxidants is important, Dr. Arellano-Mendoza said, because different antioxidants work differently. Some (catalase, glutathione peroxidase, and superoxide dismutase) are enzymatic, catalyzing reactions that convert free radicals to oxygen and water. Others terminate free radicals by preventing the propagation of oxidative chain reactions (vitamins A and C, flavonoids, uric acid, bilirubin, albumin, and members of the thiol group). A third group consists of metal-binding proteins that sequester free iron or copper to prevent free radical production (ferritin, transferrin, lactoferrin, and ceruloplasmin).

“I think we can now consider antioxidants a part of the tools we use in treating some pigmentary disorders as melasma,” she said. “We need to choose the compounds carefully, and we definitely need more in vivo research, but the findings are very encouraging.”

Dr. Arellano-Mendoza had no relevant financial disclosures.

This article was updated 3/20/17.
 

[email protected]

On Twitter @Alz_Gal

ORLANDO – A two-step regimen of high-potency topical antioxidants followed by a mineral-based sunscreen may help repair light-induced skin damage and protect against new damage in patients with melasma.

Recent studies suggest that the antioxidants tamp down inflammatory cytokines and damage of oxidative stress, Maria Ivonne Arellano-Mendoza, MD, said during a special focus session on Latino skin held at the annual meeting of the American Academy of Dermatology.

“Many compounds are being studied for this purpose,” said Dr. Arellano-Mendoza, head of the dermatology department of the General Hospital of Mexico, Mexico City. “One combination is vitamin C, vitamin E, ubiquinone, and grape-seed extract. This effectively prevented infrared-A radiation–induced matrix metalloproteinase-1 messenger RNA expression in human skin. This combination can be found now in some sunscreens and daily care products.”

Another effective combination seems to be a mixture of ferulic acid, tocopherol, and vitamin C, she said.

The two-step process of regularly using a topical product with antioxidants before applying a sunscreen is all there is for now, she added, because so far it’s been impossible to combine the agents in a single product.

“The challenge will be how to create a product that stays on the surface of the skin to protect it from light, while liberating the antioxidants to penetrate the skin,” she commented.

The antioxidants’ benefits, however, will be obliterated by the continued effects of some light wavelengths that aggravate melasma unless they are used in sequence with a light-scattering sunscreen.

Sunscreens are critical components of a melasma treatment regimen, Dr. Arellano-Mendoza said. “Sunscreens are a cornerstone of treatment. We clearly tell our patients that sunscreens have to be used every day, forever, and if they are not used properly, they will have no improvement.”

Patients with hyperpigmentation disorders are susceptible to longer wavelengths that aren’t covered by chemically derived sunscreens. Longer wavelengths, including infrared light and visible light, have been shown to increase expression of matrix metalloproteinase (MMP) -1 and -9, decrease expression of type 1 procollagen, and can induce macrophage infiltration. These wavelengths also increase reactive oxygen species and proinflammatory cytokines in vitro, Dr. Arellano-Mendoza noted.

Visible light can cause erythema, transient and long-lasting hyperpigmentation, thermal damage, free radical production, and premature photoaging. It also can stimulate the production of reactive oxygen species that can damage DNA.

Mineral-based, inorganic sunscreens, however – like those with titanium dioxide, zinc oxide, and iron oxide – scatter all wavelengths.

“These micronized forms of metal oxides not only scatter and reflect light, they also absorb ultraviolet radiation. The compounds aren’t new,” she said. In 1991, Dr. Elaine Kaye of Harvard University, Boston, and associates described (Arch Dermatol. 1991;127:351-5) opaque physical sunscreens that were useful blockers of visible light and found that transmittance of light can be lowered by adding iron oxide, Dr. Arellano-Mendoza pointed out.

The inorganic sunscreens have never been widely adopted because they are highly pigmented with white or, in the case of iron oxide, with red. “Not many people accepted [the iron-containing compounds] because of the redness, but now different shades are going to be hitting the market soon,” and the hope is that consumers will find them more appealing, she said.

This is good news, as the data emerging around iron oxide are intriguingly positive. A 2015 study showed that a sunscreen with iron oxides prevented melasma relapse during the summer months. Patients were randomized to the same ultraviolet filter topical sunscreen, but for one group, micronized iron oxide was added to it. After 6 months, the median melasma area severity index score was significantly better in the group using the iron oxide compound (J Am Acad Dermatol. 2015 Jan;72[1]:189-90.e1).

Support for the two-step regimen appeared in 2014, when a small study randomized 30 healthy volunteers to an SPF 30 sunscreen or the same sunscreen supplemented with an antioxidant cocktail of grape seed extract, vitamin E, ubiquinone, and vitamin C. The endpoint was MMP-1 upregulation after exposure to infrared-A light. Skin treated with the combination regimen showed significantly lower MMP-1 activation, leading the authors to conclude that the combination of topical antioxidants conferred protection against the irradiation (Photochem Photobiol. 2015 Jan-Feb;91[1]:248-50).

Those same authors published a companion article (Photodermatol Photoimmunol Photomed. 2014;30:167-74) suggesting that another antioxidant mixture (ferulic acid, tocopherol, and vitamin C) was similarly effective.

Using a combination of antioxidants is important, Dr. Arellano-Mendoza said, because different antioxidants work differently. Some (catalase, glutathione peroxidase, and superoxide dismutase) are enzymatic, catalyzing reactions that convert free radicals to oxygen and water. Others terminate free radicals by preventing the propagation of oxidative chain reactions (vitamins A and C, flavonoids, uric acid, bilirubin, albumin, and members of the thiol group). A third group consists of metal-binding proteins that sequester free iron or copper to prevent free radical production (ferritin, transferrin, lactoferrin, and ceruloplasmin).

“I think we can now consider antioxidants a part of the tools we use in treating some pigmentary disorders as melasma,” she said. “We need to choose the compounds carefully, and we definitely need more in vivo research, but the findings are very encouraging.”

Dr. Arellano-Mendoza had no relevant financial disclosures.

This article was updated 3/20/17.
 

[email protected]

On Twitter @Alz_Gal

The Diagnosis: Erythema Dyschromicum Perstans

Erythema dyschromicum perstans (EDP), also referred to as ashy dermatosis, was first described by Ramirez¹ in 1957 who labeled the patients los cenicientos (the ashen ones). It preferentially affects women in the second decade of life; however, patients of all ages can be affected, with reported cases occurring in children as young as 2 years of age.² Most patients have Fitzpatrick skin type IV, mainly Amerindian, Hispanic South Asian, and Southwest Asian; however, there are cases reported worldwide.³ A genetic predisposition is proposed, as major histocompatibility complex genes associated with HLA-DR4⁎0407 are frequent in Mexican patients with ashy dermatosis and in the Amerindian population.⁴

The etiology of EDP is unknown. Various contributing factors have been reported including alimentary, occupational, and climatic factors,^5,6 yet none have been conclusively demonstrated. High expression of CD36 (thrombospondin receptor not found in normal skin) in spinous and granular layers, CD94 (cytotoxic cell marker) in the basal cell layer and in the inflammatory dermal infiltrate,⁷ and focal keratinocytic expression of intercellular adhesion molecule I (CD54) in the active lesions of EDP, as well as the absence of these findings in normal skin, suggests an immunologic role in the development of the disease.⁸

Erythema dyschromicum perstans presents clinically with blue-gray hyperpigmented macules varying in size and shape and developing symmetrically in both sun-exposed and sun-protected areas of the face, neck, trunk, arms, and sometimes the dorsal hands (Figures 1 and 2). Notable sparing of the palms, soles, scalp, and mucous membranes occurs.

Occasionally, in the early active stage of the disease, elevated erythematous borders are noted surrounding the hyperpigmented macules. Eventually a hypopigmented halo develops after a prolonged duration of disease.⁹ The eruption typically is chronic and asymptomatic, though some cases may be pruritic.¹⁰

Histopathologically, the early lesions of EDP with an erythematous active border reveal lichenoid dermatitis with basal vacuolar change and occasional Civatte bodies. A mild to moderate perivascular lymphohistiocytic infiltrate admixed with melanophages can be seen in the papillary dermis (Figure 3). In older lesions, the inflammatory infiltrate is sparse, and pigment incontinence consistent with postinflammatory pigmentation is prominent, though melanophages extending deep into the reticular dermis may aid in distinguishing EDP from other causes of postinflammatory pigment alteration.^7,11

Erythema dyschromicum perstans and lichen planus pigmentosus (LPP) may be indistinguishable histopathologically and may both be variants of lichen planus actinicus. Lichen planus pigmentosus often differs from EDP in that it presents with brown-black macules and patches often on the face and flexural areas. A subset of cases of LPP also may have mucous membrane involvement. The erythematous border that characterizes the active lesion of EDP is characteristically absent in LPP. In addition, pruritus often is reported with LPP. Direct immunofluorescence is not a beneficial tool in distinguishing the entities.¹²

Other differential diagnoses of predominantly facial hyperpigmentation include a lichenoid drug eruption; drug-induced hyperpigmentation (deposition disorder); postinflammatory hyperpigmentation following atopic dermatitis; contact dermatitis or photosensitivity reaction; early pinta; and cutaneous findings of systemic diseases manifesting with diffuse hyperpigmentation such as lupus erythematosus, dermatomyositis, hemochromatosis, and Addison disease. A detailed history including medication use, thorough clinical examination, and careful histopathologic evaluation will help distinguish these conditions.

Chrysiasis is a rare bluish to slate gray discoloration of the skin that predominantly occurs in sun-exposed areas. It is caused by chronic use of gold salts, which have been used to treat rheumatoid arthritis. UV light may contribute to induce the uptake of gold and subsequently stimulate tyrosinase activity.¹³ Histologic features of chrysiasis include dermal and perivascular gold deposition within the macrophages and endothelial cells as well as extracellular granules. It demonstrates an orange-red birefringence on fluorescent microscopy.^14,15

Minocycline-induced hyperpigmentation is a well-recognized side effect of this drug. It is dose dependent and appears as a blue-black pigmentation that most frequently affects the shins, ankles, and arms.¹⁶ Three distinct types were documented: abnormal discoloration of the skin that has been linked to deposition of pigmented metabolites of minocycline producing blue-black pigmentation at the site of scarring or prior inflammation (type 1); blue-gray pigmentation affecting normal skin, mainly the legs (type 2); and elevated levels of melanin on the sun-exposed areas producing dirty skin syndrome (type 3).^17,18

Topical and systemic corticosteroids, UV light therapy, oral dapsone, griseofulvin, retinoids, and clofazimine are reported as treatment options for ashy dermatosis, though results typically are disappointing.⁷

The Diagnosis: Erythema Dyschromicum Perstans

Erythema dyschromicum perstans (EDP), also referred to as ashy dermatosis, was first described by Ramirez¹ in 1957 who labeled the patients los cenicientos (the ashen ones). It preferentially affects women in the second decade of life; however, patients of all ages can be affected, with reported cases occurring in children as young as 2 years of age.² Most patients have Fitzpatrick skin type IV, mainly Amerindian, Hispanic South Asian, and Southwest Asian; however, there are cases reported worldwide.³ A genetic predisposition is proposed, as major histocompatibility complex genes associated with HLA-DR4⁎0407 are frequent in Mexican patients with ashy dermatosis and in the Amerindian population.⁴

The etiology of EDP is unknown. Various contributing factors have been reported including alimentary, occupational, and climatic factors,^5,6 yet none have been conclusively demonstrated. High expression of CD36 (thrombospondin receptor not found in normal skin) in spinous and granular layers, CD94 (cytotoxic cell marker) in the basal cell layer and in the inflammatory dermal infiltrate,⁷ and focal keratinocytic expression of intercellular adhesion molecule I (CD54) in the active lesions of EDP, as well as the absence of these findings in normal skin, suggests an immunologic role in the development of the disease.⁸

Erythema dyschromicum perstans presents clinically with blue-gray hyperpigmented macules varying in size and shape and developing symmetrically in both sun-exposed and sun-protected areas of the face, neck, trunk, arms, and sometimes the dorsal hands (Figures 1 and 2). Notable sparing of the palms, soles, scalp, and mucous membranes occurs.

Occasionally, in the early active stage of the disease, elevated erythematous borders are noted surrounding the hyperpigmented macules. Eventually a hypopigmented halo develops after a prolonged duration of disease.⁹ The eruption typically is chronic and asymptomatic, though some cases may be pruritic.¹⁰

Histopathologically, the early lesions of EDP with an erythematous active border reveal lichenoid dermatitis with basal vacuolar change and occasional Civatte bodies. A mild to moderate perivascular lymphohistiocytic infiltrate admixed with melanophages can be seen in the papillary dermis (Figure 3). In older lesions, the inflammatory infiltrate is sparse, and pigment incontinence consistent with postinflammatory pigmentation is prominent, though melanophages extending deep into the reticular dermis may aid in distinguishing EDP from other causes of postinflammatory pigment alteration.^7,11

Erythema dyschromicum perstans and lichen planus pigmentosus (LPP) may be indistinguishable histopathologically and may both be variants of lichen planus actinicus. Lichen planus pigmentosus often differs from EDP in that it presents with brown-black macules and patches often on the face and flexural areas. A subset of cases of LPP also may have mucous membrane involvement. The erythematous border that characterizes the active lesion of EDP is characteristically absent in LPP. In addition, pruritus often is reported with LPP. Direct immunofluorescence is not a beneficial tool in distinguishing the entities.¹²

Other differential diagnoses of predominantly facial hyperpigmentation include a lichenoid drug eruption; drug-induced hyperpigmentation (deposition disorder); postinflammatory hyperpigmentation following atopic dermatitis; contact dermatitis or photosensitivity reaction; early pinta; and cutaneous findings of systemic diseases manifesting with diffuse hyperpigmentation such as lupus erythematosus, dermatomyositis, hemochromatosis, and Addison disease. A detailed history including medication use, thorough clinical examination, and careful histopathologic evaluation will help distinguish these conditions.

Chrysiasis is a rare bluish to slate gray discoloration of the skin that predominantly occurs in sun-exposed areas. It is caused by chronic use of gold salts, which have been used to treat rheumatoid arthritis. UV light may contribute to induce the uptake of gold and subsequently stimulate tyrosinase activity.¹³ Histologic features of chrysiasis include dermal and perivascular gold deposition within the macrophages and endothelial cells as well as extracellular granules. It demonstrates an orange-red birefringence on fluorescent microscopy.^14,15

Minocycline-induced hyperpigmentation is a well-recognized side effect of this drug. It is dose dependent and appears as a blue-black pigmentation that most frequently affects the shins, ankles, and arms.¹⁶ Three distinct types were documented: abnormal discoloration of the skin that has been linked to deposition of pigmented metabolites of minocycline producing blue-black pigmentation at the site of scarring or prior inflammation (type 1); blue-gray pigmentation affecting normal skin, mainly the legs (type 2); and elevated levels of melanin on the sun-exposed areas producing dirty skin syndrome (type 3).^17,18

Topical and systemic corticosteroids, UV light therapy, oral dapsone, griseofulvin, retinoids, and clofazimine are reported as treatment options for ashy dermatosis, though results typically are disappointing.⁷

The Diagnosis: Erythema Dyschromicum Perstans

Erythema dyschromicum perstans (EDP), also referred to as ashy dermatosis, was first described by Ramirez¹ in 1957 who labeled the patients los cenicientos (the ashen ones). It preferentially affects women in the second decade of life; however, patients of all ages can be affected, with reported cases occurring in children as young as 2 years of age.² Most patients have Fitzpatrick skin type IV, mainly Amerindian, Hispanic South Asian, and Southwest Asian; however, there are cases reported worldwide.³ A genetic predisposition is proposed, as major histocompatibility complex genes associated with HLA-DR4⁎0407 are frequent in Mexican patients with ashy dermatosis and in the Amerindian population.⁴

The etiology of EDP is unknown. Various contributing factors have been reported including alimentary, occupational, and climatic factors,^5,6 yet none have been conclusively demonstrated. High expression of CD36 (thrombospondin receptor not found in normal skin) in spinous and granular layers, CD94 (cytotoxic cell marker) in the basal cell layer and in the inflammatory dermal infiltrate,⁷ and focal keratinocytic expression of intercellular adhesion molecule I (CD54) in the active lesions of EDP, as well as the absence of these findings in normal skin, suggests an immunologic role in the development of the disease.⁸

Erythema dyschromicum perstans presents clinically with blue-gray hyperpigmented macules varying in size and shape and developing symmetrically in both sun-exposed and sun-protected areas of the face, neck, trunk, arms, and sometimes the dorsal hands (Figures 1 and 2). Notable sparing of the palms, soles, scalp, and mucous membranes occurs.

Occasionally, in the early active stage of the disease, elevated erythematous borders are noted surrounding the hyperpigmented macules. Eventually a hypopigmented halo develops after a prolonged duration of disease.⁹ The eruption typically is chronic and asymptomatic, though some cases may be pruritic.¹⁰

Histopathologically, the early lesions of EDP with an erythematous active border reveal lichenoid dermatitis with basal vacuolar change and occasional Civatte bodies. A mild to moderate perivascular lymphohistiocytic infiltrate admixed with melanophages can be seen in the papillary dermis (Figure 3). In older lesions, the inflammatory infiltrate is sparse, and pigment incontinence consistent with postinflammatory pigmentation is prominent, though melanophages extending deep into the reticular dermis may aid in distinguishing EDP from other causes of postinflammatory pigment alteration.^7,11

Erythema dyschromicum perstans and lichen planus pigmentosus (LPP) may be indistinguishable histopathologically and may both be variants of lichen planus actinicus. Lichen planus pigmentosus often differs from EDP in that it presents with brown-black macules and patches often on the face and flexural areas. A subset of cases of LPP also may have mucous membrane involvement. The erythematous border that characterizes the active lesion of EDP is characteristically absent in LPP. In addition, pruritus often is reported with LPP. Direct immunofluorescence is not a beneficial tool in distinguishing the entities.¹²

Other differential diagnoses of predominantly facial hyperpigmentation include a lichenoid drug eruption; drug-induced hyperpigmentation (deposition disorder); postinflammatory hyperpigmentation following atopic dermatitis; contact dermatitis or photosensitivity reaction; early pinta; and cutaneous findings of systemic diseases manifesting with diffuse hyperpigmentation such as lupus erythematosus, dermatomyositis, hemochromatosis, and Addison disease. A detailed history including medication use, thorough clinical examination, and careful histopathologic evaluation will help distinguish these conditions.

Chrysiasis is a rare bluish to slate gray discoloration of the skin that predominantly occurs in sun-exposed areas. It is caused by chronic use of gold salts, which have been used to treat rheumatoid arthritis. UV light may contribute to induce the uptake of gold and subsequently stimulate tyrosinase activity.¹³ Histologic features of chrysiasis include dermal and perivascular gold deposition within the macrophages and endothelial cells as well as extracellular granules. It demonstrates an orange-red birefringence on fluorescent microscopy.^14,15

Minocycline-induced hyperpigmentation is a well-recognized side effect of this drug. It is dose dependent and appears as a blue-black pigmentation that most frequently affects the shins, ankles, and arms.¹⁶ Three distinct types were documented: abnormal discoloration of the skin that has been linked to deposition of pigmented metabolites of minocycline producing blue-black pigmentation at the site of scarring or prior inflammation (type 1); blue-gray pigmentation affecting normal skin, mainly the legs (type 2); and elevated levels of melanin on the sun-exposed areas producing dirty skin syndrome (type 3).^17,18

Topical and systemic corticosteroids, UV light therapy, oral dapsone, griseofulvin, retinoids, and clofazimine are reported as treatment options for ashy dermatosis, though results typically are disappointing.⁷

To the Editor:

Vascular lesions associated with melanocytic nevi were first described by Ota et al¹ in 1947 and given the name phacomatosis pigmentovascularis. In 2005, Happle² reclassified phacomatosis pigmentovascularis into 3 well-defined types: (1) phacomatosis cesioflammea: blue spots (caesius means bluish gray in Latin) and nevus flammeus; (2) phacomatosis spilorosea: nevus spilus coexisting with a pale pink telangiectatic nevus; and (3) phacomatosis cesiomarmorata: blue spots and cutis marmorata telangiectatica congenita. In 2011 Joshi et al³ described a case of a 31-year-old woman who had a port-wine stain in association with neurofibromatosis type I (NF-1). We present a case of phacomatosis cesioflammea in association with NF-1.

A 20-year-old woman presented to our outpatient section with a bluish black birthmark on the left side of the face since birth with the onset of multiple painless flesh-colored nodules on the trunk and arms of 1 year’s duration. She reported having occasional pruritus over the nodular lesions. Cutaneous examination showed multiple well-defined café au lait macules (0.5–3.0 cm) with regular margins. Multiple flesh-colored nodules were evident on the upper arms (Figure 1) and trunk. The nodules were firm in consistency and showed buttonholing phenomenon with some of the lesions demonstrating bag-of-worms consistency on palpation. Both palms showed multiple brownish frecklelike macules (Figure 2). A single bluish patch extended from the left ala of the nose to the sideburns. Adjoining the bluish patch was a subtle, ill-defined, nonblanchable red patch extending from the lower margin of the bluish patch to the mandibular ridge (Figure 3). Ocular examination showed melanosis bulbi of the left sclera and a few iris hamartomas (Lisch nodules) in both eyes. A biopsy of the skin nodule was obtained under local anesthesia after obtaining the patient’s informed consent; the specimen was fixed in 10% buffered formalin. A hematoxylin and eosin–stained section showed a well-circumscribed nonencapsulated tumor in the dermis composed of loosely spaced spindle cells and wavy collagenous strands (Figure 4). Routine hemogram and blood biochemistry including urinalysis were within reference range. Radiologic examination of the long bones was unremarkable. Our patient had 3 of 6 criteria defined by the National Institutes of Health for diagnosis of NF-1.⁴ On clinicopathological correlation we made a diagnosis of phacomatosis cesioflammea in association with NF-1. We have reassured the patient about the benign nature of vascular nevus. She was informed that the skin nodules could increase in size during pregnancy and to regularly follow-up with an eye specialist if any visual abnormalities occur.

The term phacomatosis is applied to genetically determined disorders of tissue derived from ectodermal origin (eg, skin, central nervous system, eyes) and commonly includes NF-1, tuberous sclerosis, and von Hippel-Lindau syndrome. Neurofibromatosis type I was first described by German pathologist Friedrich Daniel von Recklinghausen.⁵ Phacomatosis pigmentovascularis has been defined as the association of vascular nevus with a pigmentary nevus. Its pathogenesis can be explained by the twin spotting phenomenon.⁶ Twin spots are paired patches of mutant tissue that differ from each other and from the surrounding normal background skin. They can occur as 2 clinical types: allelic and nonallelic twin spotting. Our patient had nonallelic twin spots for 2 nevoid conditions: vascular (nevus flammeus) and pigmentary (nevus of Ota). Nevus of Ota was distributed in the V2 segment (maxillary nerve) of the fifth cranial nerve along with classical melanosis bulbi, which is considered a characteristic clinical feature of nevus of Ota (nevus cesius).⁷ Nevus flammeus (port-wine stain) is a vascular malformation presenting with flat lesions that persists throughout a patient’s life. The phenomenon of twin spotting, or didymosis (didymos means twin in Greek), has been proposed for co-occurrence of vascular and pigmented nevi.⁸ The association of NF-1 along with phacomatosis cesioflammea (a twin spot) could be explained from mosaicism of tissues derived from neuroectodermal and mesenchymal elements. Neurofibromatosis type I can occur as a mosaic disorder due to either postzygotic germ line or somatic mutations in the NF1 gene located on the proximal long arm of chromosome 17.⁹ Irrespective of the mutational event, a mosaic patient has a mixture of cells, some have normal copies of a particular gene and others have an abnormal copy of the same gene. Somatic mutation can lead to segmental (localized), generalized, or gonadal mosaicism. Somatic mutations occurring early during embryonic development produce generalized mosaicism, and generalized mosaics clinically appear similar to nonmosaic NF-1 cases.^10,11 However, due to a lack of adequate facilities for mutation analysis and financial constraints, we were unable to confirm our case as generalized somatic mosaic for NF1 gene.

Several morphologic abnormalities have been reported with phacomatosis cesioflammea. Wu et al¹² reported a single case of phacomatosis cesioflammea associated with pectus excavatum in a 9-month-old infant. Shields et al¹³ suggested that a thorough ocular examination on a periodic basis is essential to rule out melanoma of ocular tissues in patients with nevus flammeus and ocular melanosis.

Phacomatosis cesioflammea can occur in association with NF-1. The exact incidence of association is not known. The nevoid condition can be treated with appropriate lasers.

To the Editor:

Vascular lesions associated with melanocytic nevi were first described by Ota et al¹ in 1947 and given the name phacomatosis pigmentovascularis. In 2005, Happle² reclassified phacomatosis pigmentovascularis into 3 well-defined types: (1) phacomatosis cesioflammea: blue spots (caesius means bluish gray in Latin) and nevus flammeus; (2) phacomatosis spilorosea: nevus spilus coexisting with a pale pink telangiectatic nevus; and (3) phacomatosis cesiomarmorata: blue spots and cutis marmorata telangiectatica congenita. In 2011 Joshi et al³ described a case of a 31-year-old woman who had a port-wine stain in association with neurofibromatosis type I (NF-1). We present a case of phacomatosis cesioflammea in association with NF-1.

A 20-year-old woman presented to our outpatient section with a bluish black birthmark on the left side of the face since birth with the onset of multiple painless flesh-colored nodules on the trunk and arms of 1 year’s duration. She reported having occasional pruritus over the nodular lesions. Cutaneous examination showed multiple well-defined café au lait macules (0.5–3.0 cm) with regular margins. Multiple flesh-colored nodules were evident on the upper arms (Figure 1) and trunk. The nodules were firm in consistency and showed buttonholing phenomenon with some of the lesions demonstrating bag-of-worms consistency on palpation. Both palms showed multiple brownish frecklelike macules (Figure 2). A single bluish patch extended from the left ala of the nose to the sideburns. Adjoining the bluish patch was a subtle, ill-defined, nonblanchable red patch extending from the lower margin of the bluish patch to the mandibular ridge (Figure 3). Ocular examination showed melanosis bulbi of the left sclera and a few iris hamartomas (Lisch nodules) in both eyes. A biopsy of the skin nodule was obtained under local anesthesia after obtaining the patient’s informed consent; the specimen was fixed in 10% buffered formalin. A hematoxylin and eosin–stained section showed a well-circumscribed nonencapsulated tumor in the dermis composed of loosely spaced spindle cells and wavy collagenous strands (Figure 4). Routine hemogram and blood biochemistry including urinalysis were within reference range. Radiologic examination of the long bones was unremarkable. Our patient had 3 of 6 criteria defined by the National Institutes of Health for diagnosis of NF-1.⁴ On clinicopathological correlation we made a diagnosis of phacomatosis cesioflammea in association with NF-1. We have reassured the patient about the benign nature of vascular nevus. She was informed that the skin nodules could increase in size during pregnancy and to regularly follow-up with an eye specialist if any visual abnormalities occur.

The term phacomatosis is applied to genetically determined disorders of tissue derived from ectodermal origin (eg, skin, central nervous system, eyes) and commonly includes NF-1, tuberous sclerosis, and von Hippel-Lindau syndrome. Neurofibromatosis type I was first described by German pathologist Friedrich Daniel von Recklinghausen.⁵ Phacomatosis pigmentovascularis has been defined as the association of vascular nevus with a pigmentary nevus. Its pathogenesis can be explained by the twin spotting phenomenon.⁶ Twin spots are paired patches of mutant tissue that differ from each other and from the surrounding normal background skin. They can occur as 2 clinical types: allelic and nonallelic twin spotting. Our patient had nonallelic twin spots for 2 nevoid conditions: vascular (nevus flammeus) and pigmentary (nevus of Ota). Nevus of Ota was distributed in the V2 segment (maxillary nerve) of the fifth cranial nerve along with classical melanosis bulbi, which is considered a characteristic clinical feature of nevus of Ota (nevus cesius).⁷ Nevus flammeus (port-wine stain) is a vascular malformation presenting with flat lesions that persists throughout a patient’s life. The phenomenon of twin spotting, or didymosis (didymos means twin in Greek), has been proposed for co-occurrence of vascular and pigmented nevi.⁸ The association of NF-1 along with phacomatosis cesioflammea (a twin spot) could be explained from mosaicism of tissues derived from neuroectodermal and mesenchymal elements. Neurofibromatosis type I can occur as a mosaic disorder due to either postzygotic germ line or somatic mutations in the NF1 gene located on the proximal long arm of chromosome 17.⁹ Irrespective of the mutational event, a mosaic patient has a mixture of cells, some have normal copies of a particular gene and others have an abnormal copy of the same gene. Somatic mutation can lead to segmental (localized), generalized, or gonadal mosaicism. Somatic mutations occurring early during embryonic development produce generalized mosaicism, and generalized mosaics clinically appear similar to nonmosaic NF-1 cases.^10,11 However, due to a lack of adequate facilities for mutation analysis and financial constraints, we were unable to confirm our case as generalized somatic mosaic for NF1 gene.

Several morphologic abnormalities have been reported with phacomatosis cesioflammea. Wu et al¹² reported a single case of phacomatosis cesioflammea associated with pectus excavatum in a 9-month-old infant. Shields et al¹³ suggested that a thorough ocular examination on a periodic basis is essential to rule out melanoma of ocular tissues in patients with nevus flammeus and ocular melanosis.

Phacomatosis cesioflammea can occur in association with NF-1. The exact incidence of association is not known. The nevoid condition can be treated with appropriate lasers.

To the Editor:

Vascular lesions associated with melanocytic nevi were first described by Ota et al¹ in 1947 and given the name phacomatosis pigmentovascularis. In 2005, Happle² reclassified phacomatosis pigmentovascularis into 3 well-defined types: (1) phacomatosis cesioflammea: blue spots (caesius means bluish gray in Latin) and nevus flammeus; (2) phacomatosis spilorosea: nevus spilus coexisting with a pale pink telangiectatic nevus; and (3) phacomatosis cesiomarmorata: blue spots and cutis marmorata telangiectatica congenita. In 2011 Joshi et al³ described a case of a 31-year-old woman who had a port-wine stain in association with neurofibromatosis type I (NF-1). We present a case of phacomatosis cesioflammea in association with NF-1.

A 20-year-old woman presented to our outpatient section with a bluish black birthmark on the left side of the face since birth with the onset of multiple painless flesh-colored nodules on the trunk and arms of 1 year’s duration. She reported having occasional pruritus over the nodular lesions. Cutaneous examination showed multiple well-defined café au lait macules (0.5–3.0 cm) with regular margins. Multiple flesh-colored nodules were evident on the upper arms (Figure 1) and trunk. The nodules were firm in consistency and showed buttonholing phenomenon with some of the lesions demonstrating bag-of-worms consistency on palpation. Both palms showed multiple brownish frecklelike macules (Figure 2). A single bluish patch extended from the left ala of the nose to the sideburns. Adjoining the bluish patch was a subtle, ill-defined, nonblanchable red patch extending from the lower margin of the bluish patch to the mandibular ridge (Figure 3). Ocular examination showed melanosis bulbi of the left sclera and a few iris hamartomas (Lisch nodules) in both eyes. A biopsy of the skin nodule was obtained under local anesthesia after obtaining the patient’s informed consent; the specimen was fixed in 10% buffered formalin. A hematoxylin and eosin–stained section showed a well-circumscribed nonencapsulated tumor in the dermis composed of loosely spaced spindle cells and wavy collagenous strands (Figure 4). Routine hemogram and blood biochemistry including urinalysis were within reference range. Radiologic examination of the long bones was unremarkable. Our patient had 3 of 6 criteria defined by the National Institutes of Health for diagnosis of NF-1.⁴ On clinicopathological correlation we made a diagnosis of phacomatosis cesioflammea in association with NF-1. We have reassured the patient about the benign nature of vascular nevus. She was informed that the skin nodules could increase in size during pregnancy and to regularly follow-up with an eye specialist if any visual abnormalities occur.

The term phacomatosis is applied to genetically determined disorders of tissue derived from ectodermal origin (eg, skin, central nervous system, eyes) and commonly includes NF-1, tuberous sclerosis, and von Hippel-Lindau syndrome. Neurofibromatosis type I was first described by German pathologist Friedrich Daniel von Recklinghausen.⁵ Phacomatosis pigmentovascularis has been defined as the association of vascular nevus with a pigmentary nevus. Its pathogenesis can be explained by the twin spotting phenomenon.⁶ Twin spots are paired patches of mutant tissue that differ from each other and from the surrounding normal background skin. They can occur as 2 clinical types: allelic and nonallelic twin spotting. Our patient had nonallelic twin spots for 2 nevoid conditions: vascular (nevus flammeus) and pigmentary (nevus of Ota). Nevus of Ota was distributed in the V2 segment (maxillary nerve) of the fifth cranial nerve along with classical melanosis bulbi, which is considered a characteristic clinical feature of nevus of Ota (nevus cesius).⁷ Nevus flammeus (port-wine stain) is a vascular malformation presenting with flat lesions that persists throughout a patient’s life. The phenomenon of twin spotting, or didymosis (didymos means twin in Greek), has been proposed for co-occurrence of vascular and pigmented nevi.⁸ The association of NF-1 along with phacomatosis cesioflammea (a twin spot) could be explained from mosaicism of tissues derived from neuroectodermal and mesenchymal elements. Neurofibromatosis type I can occur as a mosaic disorder due to either postzygotic germ line or somatic mutations in the NF1 gene located on the proximal long arm of chromosome 17.⁹ Irrespective of the mutational event, a mosaic patient has a mixture of cells, some have normal copies of a particular gene and others have an abnormal copy of the same gene. Somatic mutation can lead to segmental (localized), generalized, or gonadal mosaicism. Somatic mutations occurring early during embryonic development produce generalized mosaicism, and generalized mosaics clinically appear similar to nonmosaic NF-1 cases.^10,11 However, due to a lack of adequate facilities for mutation analysis and financial constraints, we were unable to confirm our case as generalized somatic mosaic for NF1 gene.

Several morphologic abnormalities have been reported with phacomatosis cesioflammea. Wu et al¹² reported a single case of phacomatosis cesioflammea associated with pectus excavatum in a 9-month-old infant. Shields et al¹³ suggested that a thorough ocular examination on a periodic basis is essential to rule out melanoma of ocular tissues in patients with nevus flammeus and ocular melanosis.

Phacomatosis cesioflammea can occur in association with NF-1. The exact incidence of association is not known. The nevoid condition can be treated with appropriate lasers.

Upper extremity moles were associated with a 37% increase in the likelihood of vitiligo among white women, according to an analysis of the prospective Nurses’ Health Study.

“Women with a higher tanning ability and women who had a history of blistering sunburns in childhood were also found to have a higher risk of developing vitiligo,” Rachel Dunlap, MD, of the department of dermatology, Brown University in Providence, R.I., and her associates wrote in the Journal of Investigative Dermatology.

Vitiligo is the most common cutaneous depigmentation disorder, but associated risk factors are poorly understood, the investigators noted. They examined ties between skin pigmentation, reactions to sun exposure, and new onset vitiligo in the Nurses’ Health Study, a population-based prospective cohort study. Study participants were asked to report the number of moles on their left arms measuring at least 3 mm in diameter, their reactions to sunburn and ability to tan during childhood, and whether they had vitiligo diagnosed by a physician. A total of 51,337 women answered the question about moles, and 68,590 women answered the question about vitiligo, the investigators said (J Invest Dermatol. 2017 Feb 14. doi: 10.1016/j.jid.2017.02.004).

A total of 271 cases of vitiligo developed over 835,594 person-years. Women who reported at least one left arm mole larger than 3 mm were significantly more likely to report incident vitiligo, compared with women without moles (hazard ratio, 1.37; 95% confidence interval, 1.02-1.83), even after controlling for age, hair color, history of exposure to direct sunlight, skin tanning ability, and severity of reaction to sunburn. Developing an “average” tan or a “deep” tan after prolonged sun exposure also were significantly associated with vitiligo with hazard ratios of 2.28 (95% CI, 1.12-4.65) and 2.59 (95% CI, 1.21-5.54), respectively, “when compared to those who had minimal skin reactions or less severe burns when exposed to the sun,” the authors wrote.

A history of at least one blistering sunburn after 2 hours of sun exposure also predicted vitiligo (HR, 2.17; 95% CI, 1.15-4.10), while hair color did not.

“The benefits of good sun protection can be expanded to include potential vitiligo prevention, which may be particularly applicable to adult patients with vitiligo who are concerned about their children developing the condition,” the investigators commented. “Future studies will examine the incidence of other influencing factors, such as melanoma and melanoma associated leukoderma in this population.”

External funding sources included the National Institutes of Health and Dermatology Foundation. The investigators reported having no conflicts of interest.

Upper extremity moles were associated with a 37% increase in the likelihood of vitiligo among white women, according to an analysis of the prospective Nurses’ Health Study.

“Women with a higher tanning ability and women who had a history of blistering sunburns in childhood were also found to have a higher risk of developing vitiligo,” Rachel Dunlap, MD, of the department of dermatology, Brown University in Providence, R.I., and her associates wrote in the Journal of Investigative Dermatology.

Vitiligo is the most common cutaneous depigmentation disorder, but associated risk factors are poorly understood, the investigators noted. They examined ties between skin pigmentation, reactions to sun exposure, and new onset vitiligo in the Nurses’ Health Study, a population-based prospective cohort study. Study participants were asked to report the number of moles on their left arms measuring at least 3 mm in diameter, their reactions to sunburn and ability to tan during childhood, and whether they had vitiligo diagnosed by a physician. A total of 51,337 women answered the question about moles, and 68,590 women answered the question about vitiligo, the investigators said (J Invest Dermatol. 2017 Feb 14. doi: 10.1016/j.jid.2017.02.004).

A total of 271 cases of vitiligo developed over 835,594 person-years. Women who reported at least one left arm mole larger than 3 mm were significantly more likely to report incident vitiligo, compared with women without moles (hazard ratio, 1.37; 95% confidence interval, 1.02-1.83), even after controlling for age, hair color, history of exposure to direct sunlight, skin tanning ability, and severity of reaction to sunburn. Developing an “average” tan or a “deep” tan after prolonged sun exposure also were significantly associated with vitiligo with hazard ratios of 2.28 (95% CI, 1.12-4.65) and 2.59 (95% CI, 1.21-5.54), respectively, “when compared to those who had minimal skin reactions or less severe burns when exposed to the sun,” the authors wrote.

A history of at least one blistering sunburn after 2 hours of sun exposure also predicted vitiligo (HR, 2.17; 95% CI, 1.15-4.10), while hair color did not.

“The benefits of good sun protection can be expanded to include potential vitiligo prevention, which may be particularly applicable to adult patients with vitiligo who are concerned about their children developing the condition,” the investigators commented. “Future studies will examine the incidence of other influencing factors, such as melanoma and melanoma associated leukoderma in this population.”

External funding sources included the National Institutes of Health and Dermatology Foundation. The investigators reported having no conflicts of interest.

Upper extremity moles were associated with a 37% increase in the likelihood of vitiligo among white women, according to an analysis of the prospective Nurses’ Health Study.

“Women with a higher tanning ability and women who had a history of blistering sunburns in childhood were also found to have a higher risk of developing vitiligo,” Rachel Dunlap, MD, of the department of dermatology, Brown University in Providence, R.I., and her associates wrote in the Journal of Investigative Dermatology.

Vitiligo is the most common cutaneous depigmentation disorder, but associated risk factors are poorly understood, the investigators noted. They examined ties between skin pigmentation, reactions to sun exposure, and new onset vitiligo in the Nurses’ Health Study, a population-based prospective cohort study. Study participants were asked to report the number of moles on their left arms measuring at least 3 mm in diameter, their reactions to sunburn and ability to tan during childhood, and whether they had vitiligo diagnosed by a physician. A total of 51,337 women answered the question about moles, and 68,590 women answered the question about vitiligo, the investigators said (J Invest Dermatol. 2017 Feb 14. doi: 10.1016/j.jid.2017.02.004).

A total of 271 cases of vitiligo developed over 835,594 person-years. Women who reported at least one left arm mole larger than 3 mm were significantly more likely to report incident vitiligo, compared with women without moles (hazard ratio, 1.37; 95% confidence interval, 1.02-1.83), even after controlling for age, hair color, history of exposure to direct sunlight, skin tanning ability, and severity of reaction to sunburn. Developing an “average” tan or a “deep” tan after prolonged sun exposure also were significantly associated with vitiligo with hazard ratios of 2.28 (95% CI, 1.12-4.65) and 2.59 (95% CI, 1.21-5.54), respectively, “when compared to those who had minimal skin reactions or less severe burns when exposed to the sun,” the authors wrote.

A history of at least one blistering sunburn after 2 hours of sun exposure also predicted vitiligo (HR, 2.17; 95% CI, 1.15-4.10), while hair color did not.

“The benefits of good sun protection can be expanded to include potential vitiligo prevention, which may be particularly applicable to adult patients with vitiligo who are concerned about their children developing the condition,” the investigators commented. “Future studies will examine the incidence of other influencing factors, such as melanoma and melanoma associated leukoderma in this population.”

External funding sources included the National Institutes of Health and Dermatology Foundation. The investigators reported having no conflicts of interest.

To the Editor:

Dermatomyositis represents a rare idiopathic inflammatory process presenting with cutaneous lesions and muscular weakness. It often represents a paraneoplastic syndrome. We report the case of a 62-year-old man with a history of total-body poikiloderma and a recent diagnosis of chronic lymphocytic leukemia (CLL). Despite lacking typical features of the disease, a diagnosis of dermatomyositis was made. Our patient may represent a distinct poikilodermatous variant of dermatomyositis, sharing the generalized distribution of the erythrodermic subtype.

A 62-year-old man presented with pruritic poikiloderma involving the neck, arms, legs, abdomen, chest, and back of 2 years’ duration (Figure). He also experienced dysphagia and weakness of the legs. The rash was previously treated by other dermatologists with a combination of high-potency topical steroids and topical tacrolimus 0.1% without success. His history was notable for CLL, which had been diagnosed by a dermatologist 6 months prior to the current presentation. Prior to his visit to the dermatologist, the patient had received 6 chemotherapeutic sessions with a combination of rituximab and cyclophosphamide for the treatment of CLL. The rash did not improve with chemotherapy.

Repeat biopsies of affected regions only demonstrated features of mild interface dermatitis. Direct immunofluorescence studies showed scattered colloid body fluorescence for IgM. Because of bilateral weakness of the legs, a muscle biopsy was taken, which demonstrated severe atrophy and interstitial fibrosis, with neurogenic abnormalities detected in areas of lesser atrophy via abnormal muscle fiber–type grouping. Metabolic panel showed elevated muscle enzymes in the blood: creatine kinase, 243 U/L (reference range, 10–225 U/L); serum aldolase, 16 U/L (reference range, ≤8.1 U/L); lactate dehydrogenase, 314 U/L (reference range, 60–200 U/L). An autoimmune panel was negative for Jo-1, Scl-70, U1 ribonucleoprotein, DNA, desmoglein 1 and 3, and antiacetylcholine receptor antibodies. An elevated erythrocyte sedimentation rate was measured at 16 mm/h (reference range, 0–10 mm/h). Given these findings, the lesions were confirmed as a widespread poikilodermatous variant of dermatomyositis.

The patient was placed on a daily 50-mg dose of prednisone, which produced rapid improvement in scaling and erythema. Creatine kinase and serum aldolase levels normalized and motor strength increased. After 1 week the prednisone dosage was reduced to a daily 30-mg dose, and then 20 mg a week later. The skin lesions completely resolved within 4 to 5 months and the patient is currently on a prednisone dose of 5 mg, alternating with 2.5 mg of prednisone and rituximab infusion every 2 months.

Dermatomyositis is a rare entity with an incidence of approximately 0.5 to 1 per 100,000 individuals.¹ It presents with a characteristic rash composed of Gottron papules; pathognomonic flat violaceous papules on the dorsal interphalangeal joints, elbows, or knees; and a heliotrope rash, a violaceous erythema involving the eyelids. Poikiloderma frequently is reported to present in a shawl-like distribution, encompassing the shoulders, arms, and upper back.^1,2 Dermatomyositis of the poikilodermatous type can present in nonphotoexposed areas and photoexposed areas. The unusual feature is the total-body involvement, which is analogous to erythroderma.³

Our case may represent a distinct poikilodermatous manifestation sharing the distribution of the erythrodermic subtype. We believe that the skin lesions may have represented a paraneoplastic event presenting prior to diagnosis with CLL. Dermatomyositis has a strong association with cancer, with patients 3 times more likely to develop internal malignancy.⁴ Association is strongest for non-Hodgkin lymphoma, as well as ovarian, lung, colorectal, pancreatic, and gastric cancer. When associated with malignancy, symptoms of dermatomyositis or myositis typically precede the discovery of malignancy by an average of 1.9 years.⁵ Dermatomyositis has been previously reported to present as a paraneoplastic manifestation of CLL.⁶ One case has been reported of a patient with CLL who developed leukemia cutis presenting with poikiloderma in the characteristic dermatomyositis shawl-like distribution.⁷ The lack of dermal infiltration with leukemic cells in our patient, however, makes a paraneoplastic etiology much more likely.

Our patient’s rash did not initially improve with treatment of CLL, but dermatomyositis associated with hematological malignancy may precede, occur simultaneously, or follow the diagnosis of malignancy.⁸ Additionally, symptoms of dermatomyositis do not always parallel the course of hematological malignancy outcome. However, rituximab has been used as a treatment of dermatomyositis and may have contributed some synergistic effect in combination with prednisone in our patient.⁹

To the Editor:

Dermatomyositis represents a rare idiopathic inflammatory process presenting with cutaneous lesions and muscular weakness. It often represents a paraneoplastic syndrome. We report the case of a 62-year-old man with a history of total-body poikiloderma and a recent diagnosis of chronic lymphocytic leukemia (CLL). Despite lacking typical features of the disease, a diagnosis of dermatomyositis was made. Our patient may represent a distinct poikilodermatous variant of dermatomyositis, sharing the generalized distribution of the erythrodermic subtype.

A 62-year-old man presented with pruritic poikiloderma involving the neck, arms, legs, abdomen, chest, and back of 2 years’ duration (Figure). He also experienced dysphagia and weakness of the legs. The rash was previously treated by other dermatologists with a combination of high-potency topical steroids and topical tacrolimus 0.1% without success. His history was notable for CLL, which had been diagnosed by a dermatologist 6 months prior to the current presentation. Prior to his visit to the dermatologist, the patient had received 6 chemotherapeutic sessions with a combination of rituximab and cyclophosphamide for the treatment of CLL. The rash did not improve with chemotherapy.

Repeat biopsies of affected regions only demonstrated features of mild interface dermatitis. Direct immunofluorescence studies showed scattered colloid body fluorescence for IgM. Because of bilateral weakness of the legs, a muscle biopsy was taken, which demonstrated severe atrophy and interstitial fibrosis, with neurogenic abnormalities detected in areas of lesser atrophy via abnormal muscle fiber–type grouping. Metabolic panel showed elevated muscle enzymes in the blood: creatine kinase, 243 U/L (reference range, 10–225 U/L); serum aldolase, 16 U/L (reference range, ≤8.1 U/L); lactate dehydrogenase, 314 U/L (reference range, 60–200 U/L). An autoimmune panel was negative for Jo-1, Scl-70, U1 ribonucleoprotein, DNA, desmoglein 1 and 3, and antiacetylcholine receptor antibodies. An elevated erythrocyte sedimentation rate was measured at 16 mm/h (reference range, 0–10 mm/h). Given these findings, the lesions were confirmed as a widespread poikilodermatous variant of dermatomyositis.

The patient was placed on a daily 50-mg dose of prednisone, which produced rapid improvement in scaling and erythema. Creatine kinase and serum aldolase levels normalized and motor strength increased. After 1 week the prednisone dosage was reduced to a daily 30-mg dose, and then 20 mg a week later. The skin lesions completely resolved within 4 to 5 months and the patient is currently on a prednisone dose of 5 mg, alternating with 2.5 mg of prednisone and rituximab infusion every 2 months.

Dermatomyositis is a rare entity with an incidence of approximately 0.5 to 1 per 100,000 individuals.¹ It presents with a characteristic rash composed of Gottron papules; pathognomonic flat violaceous papules on the dorsal interphalangeal joints, elbows, or knees; and a heliotrope rash, a violaceous erythema involving the eyelids. Poikiloderma frequently is reported to present in a shawl-like distribution, encompassing the shoulders, arms, and upper back.^1,2 Dermatomyositis of the poikilodermatous type can present in nonphotoexposed areas and photoexposed areas. The unusual feature is the total-body involvement, which is analogous to erythroderma.³

Our case may represent a distinct poikilodermatous manifestation sharing the distribution of the erythrodermic subtype. We believe that the skin lesions may have represented a paraneoplastic event presenting prior to diagnosis with CLL. Dermatomyositis has a strong association with cancer, with patients 3 times more likely to develop internal malignancy.⁴ Association is strongest for non-Hodgkin lymphoma, as well as ovarian, lung, colorectal, pancreatic, and gastric cancer. When associated with malignancy, symptoms of dermatomyositis or myositis typically precede the discovery of malignancy by an average of 1.9 years.⁵ Dermatomyositis has been previously reported to present as a paraneoplastic manifestation of CLL.⁶ One case has been reported of a patient with CLL who developed leukemia cutis presenting with poikiloderma in the characteristic dermatomyositis shawl-like distribution.⁷ The lack of dermal infiltration with leukemic cells in our patient, however, makes a paraneoplastic etiology much more likely.

Our patient’s rash did not initially improve with treatment of CLL, but dermatomyositis associated with hematological malignancy may precede, occur simultaneously, or follow the diagnosis of malignancy.⁸ Additionally, symptoms of dermatomyositis do not always parallel the course of hematological malignancy outcome. However, rituximab has been used as a treatment of dermatomyositis and may have contributed some synergistic effect in combination with prednisone in our patient.⁹

To the Editor:

Dermatomyositis represents a rare idiopathic inflammatory process presenting with cutaneous lesions and muscular weakness. It often represents a paraneoplastic syndrome. We report the case of a 62-year-old man with a history of total-body poikiloderma and a recent diagnosis of chronic lymphocytic leukemia (CLL). Despite lacking typical features of the disease, a diagnosis of dermatomyositis was made. Our patient may represent a distinct poikilodermatous variant of dermatomyositis, sharing the generalized distribution of the erythrodermic subtype.

A 62-year-old man presented with pruritic poikiloderma involving the neck, arms, legs, abdomen, chest, and back of 2 years’ duration (Figure). He also experienced dysphagia and weakness of the legs. The rash was previously treated by other dermatologists with a combination of high-potency topical steroids and topical tacrolimus 0.1% without success. His history was notable for CLL, which had been diagnosed by a dermatologist 6 months prior to the current presentation. Prior to his visit to the dermatologist, the patient had received 6 chemotherapeutic sessions with a combination of rituximab and cyclophosphamide for the treatment of CLL. The rash did not improve with chemotherapy.

Repeat biopsies of affected regions only demonstrated features of mild interface dermatitis. Direct immunofluorescence studies showed scattered colloid body fluorescence for IgM. Because of bilateral weakness of the legs, a muscle biopsy was taken, which demonstrated severe atrophy and interstitial fibrosis, with neurogenic abnormalities detected in areas of lesser atrophy via abnormal muscle fiber–type grouping. Metabolic panel showed elevated muscle enzymes in the blood: creatine kinase, 243 U/L (reference range, 10–225 U/L); serum aldolase, 16 U/L (reference range, ≤8.1 U/L); lactate dehydrogenase, 314 U/L (reference range, 60–200 U/L). An autoimmune panel was negative for Jo-1, Scl-70, U1 ribonucleoprotein, DNA, desmoglein 1 and 3, and antiacetylcholine receptor antibodies. An elevated erythrocyte sedimentation rate was measured at 16 mm/h (reference range, 0–10 mm/h). Given these findings, the lesions were confirmed as a widespread poikilodermatous variant of dermatomyositis.

The patient was placed on a daily 50-mg dose of prednisone, which produced rapid improvement in scaling and erythema. Creatine kinase and serum aldolase levels normalized and motor strength increased. After 1 week the prednisone dosage was reduced to a daily 30-mg dose, and then 20 mg a week later. The skin lesions completely resolved within 4 to 5 months and the patient is currently on a prednisone dose of 5 mg, alternating with 2.5 mg of prednisone and rituximab infusion every 2 months.

Dermatomyositis is a rare entity with an incidence of approximately 0.5 to 1 per 100,000 individuals.¹ It presents with a characteristic rash composed of Gottron papules; pathognomonic flat violaceous papules on the dorsal interphalangeal joints, elbows, or knees; and a heliotrope rash, a violaceous erythema involving the eyelids. Poikiloderma frequently is reported to present in a shawl-like distribution, encompassing the shoulders, arms, and upper back.^1,2 Dermatomyositis of the poikilodermatous type can present in nonphotoexposed areas and photoexposed areas. The unusual feature is the total-body involvement, which is analogous to erythroderma.³

Our case may represent a distinct poikilodermatous manifestation sharing the distribution of the erythrodermic subtype. We believe that the skin lesions may have represented a paraneoplastic event presenting prior to diagnosis with CLL. Dermatomyositis has a strong association with cancer, with patients 3 times more likely to develop internal malignancy.⁴ Association is strongest for non-Hodgkin lymphoma, as well as ovarian, lung, colorectal, pancreatic, and gastric cancer. When associated with malignancy, symptoms of dermatomyositis or myositis typically precede the discovery of malignancy by an average of 1.9 years.⁵ Dermatomyositis has been previously reported to present as a paraneoplastic manifestation of CLL.⁶ One case has been reported of a patient with CLL who developed leukemia cutis presenting with poikiloderma in the characteristic dermatomyositis shawl-like distribution.⁷ The lack of dermal infiltration with leukemic cells in our patient, however, makes a paraneoplastic etiology much more likely.

Our patient’s rash did not initially improve with treatment of CLL, but dermatomyositis associated with hematological malignancy may precede, occur simultaneously, or follow the diagnosis of malignancy.⁸ Additionally, symptoms of dermatomyositis do not always parallel the course of hematological malignancy outcome. However, rituximab has been used as a treatment of dermatomyositis and may have contributed some synergistic effect in combination with prednisone in our patient.⁹