MDedge Dermatology

Phototherapy—particularly UVB phototherapy, which utilizes UVB rays of specific wavelengths within the UV spectrum—is indicated for a wide variety of dermatoses. In-office and at-home UVB treatments commonly are used, as are salon tanning and sunbathing. When selecting a form of phototherapy, patients are likely to consider safety, cost, effectiveness, insurance issues, and convenience. Research on patient preferences; the reasons for these preferences; and which options patients perceive to be the safest, most cost-effective, efficacious, and convenient is lacking. We aimed to assess the forms of phototherapy that patients would most consider using; the factors influencing patient preferences; and the forms patients perceived as the safest and most cost-effective, efficacious, and convenient.

Methods

Study Participants—We recruited 500 Amazon Mechanical Turk users who were 18 years or older to complete our REDCap-generated survey. The study was approved by the Wake Forest University institutional review board (Winston-Salem, North Carolina).

Evaluation—Participants were asked, “If you were diagnosed with a skin disease that benefited from UV therapy, which of the following forms of UV therapy would you consider choosing?” Participants were instructed to choose all of the forms they would consider using. Available options included in-office UV, at-home UV, home tanning, salon tanning, sunbathing, and other. Participants were asked to select which factors—from safety, cost, effectiveness, issues with insurance, convenience, and other—influenced their decision-making; which form of phototherapy they would most consider along with the factors that influenced their preference for this specific form of phototherapy; and which options they considered to be safest and most cost-effective, efficacious, and convenient. Participants were asked to provide basic sociodemographic information, level of education, income, insurance status (private, Medicare, Medicaid, Veterans Affairs, and uninsured), and distance from the nearest dermatologist.

Statistical Analysis—Descriptive and inferential statistics (χ² test) were used to analyze the data with a significance set at P<.05.

Results

Five hundred participants completed the survey (Table 1).

Factors Influencing Patient Preferences—When asked to select all forms of phototherapy they would consider, 186 (37.2%) participants selected in-office UVB, 263 (52.6%) selected at-home UV, 141 (28.2%) selected home tanning, 117 (23.4%) selected salon tanning, 191 (38.2%) selected sunbathing, and 3 (0.6%) selected other. Participants who selected in-office UVB as an option were more likely to also select salon tanning (P<.012). No other relationship was found between the UVB options and the tanning options. When asked which factors influenced their phototherapy preferences, 295 (59%) selected convenience, 266 (53.2%) selected effectiveness, 220 (44%) selected safety, 218 (43.6%) selected cost, 72 (14.4%) selected issues with insurance, and 4 (0.8%) selected other. Forms of Phototherapy Patients Consider Using—When asked which form of phototherapy they would most consider using, 179 (35.8%) participants selected at-home UVB, 108 (21.6%) selected sunbathing, 92 (18.4%) selected in-office UVB, 62 (12.4%) selected home-tanning, 57 (11.4%) selected salon tanning, 1 (0.2%) selected other, and 1 participant provided no response (P<.001).

Reasons for Using Phototherapy—Of the 179 who selected at-home UVB, 125 (70%) cited convenience as a reason. Of the 108 who selected salon tanning as their top choice, 62 (57%) cited cost as a reason. Convenience (P<.001), cost (P<.001), and safety (P=.023) were related to top preference. Issues with insurance did not have a statistically significant relationship with the top preference. However, participant insurance type was related to top phototherapy preference (P=.021), with privately insured patients more likely to select in-office UVB, whereas those with Medicaid and Medicare were more likely to select home or salon tanning. Efficacy was not related to top preference. Furthermore, age, gender, education, income, and distance from nearest dermatologist were not related to top preference.

In-office UVB was perceived to be safest (P<.001) and most efficacious (P<.001). Meanwhile, at-home UVB was selected as most convenient (P<.001). Lastly, sunbathing was determined to be most cost-effective (P<.001)(Table 2). Cost-effectiveness had a relationship (P<.001) with the participant’s insurance, as those with private insurance were more likely to select at-home UVB, whereas those with Medicare or Medicaid were more likely to select the tanning options. Additionally, of the54 uninsured participants in the survey, 29 selected sunbathing as the most cost-effective option.

Comment

Phototherapy Treatment—UVB phototherapy at a wavelength of 290 to 320 nm (311–313 nm for narrowband UVB) is used to treat various dermatoses, including psoriasis and atopic dermatitis. UVB alters skin cytokines, induces apoptosis, promotes immunosuppression, causes DNA damage, and decreases the proliferation of dendritic cells and other cells of the innate immune system.¹ In-office and at-home UV therapies make use of UVB wavelengths for treatment, while tanning and sunbathing contain not only UVB but also potentially harmful UVA rays. The wavelengths for indoor tanning devices include UVB at 280 to 315 nm and UVA at 315 to 400 nm, which are similar to those of the sun but with a different ratio of UVB to UVA and more intense total UV.² When in-office and at-home UVB options are not available, various forms of tanning such as salon tanning and sunbathing may be alternatives that are widely used.³ One of the main reasons patients consider alternative phototherapy options is cost, as 1 in-office UVB treatment may cost $140, but a month of unlimited tanning may cost $30 or perhaps nothing if a patient has a gym membership with access to a tanning bed. Lack of insurance benefits covering phototherapy can exacerbate cost burden.⁴ However, tanning beds are associated with an increased risk for melanoma and nonmelanoma cancers.^5,6 Additionally, all forms of phototherapy are associated with photoaging, but it is more intense with tanning and heliotherapy because of the presence of UVA, which penetrates deeper into the dermis.⁷ Meanwhile, for those who choose UVB therapy, deciding between an in-office and at-home UVB treatment could be a matter of convenience, as patients must consider long trips to the physician’s office; insurance status, as some insurances may not cover at-home UVB; or efficacy, which might be influenced by the presence of a physician or other medical staff. In many cases, patients may not be informed that at-home UVB is an option.

Patient Preferences—At-home UVB therapy was the most popular option in our study population, with most participants (52.6%) considering using it, and 35.9% choosing it as their top choice over all other phototherapy options. Safety, cost, and convenience were all found to be related to the option participants would most consider using. Prior analysis between at-home UVB and in-office UVB for the treatment of psoriasis determined that at-home UVB is as safe and cost-effective as in-office UVB without the inconvenience of the patient having to take time out of the week to visit the physician’s office,^8,9 making at-home UVB an option dermatologists may strongly consider for patients who value safety, cost, and convenience. Oddly, efficacy was not related to the top preference, despite being the second highest–cited factor (53.2%) for which forms of phototherapy participants would consider using. For insurance coverage, those with Medicaid and Medicare selected the cheaper tanning options with higher-than-expected frequencies. Although problems with insurance were not related to the top preference, insurance status was related, suggesting that preferences are tied to cost. Of note, while the number of dermatologists that accept Medicare has increased in the last few years, there still remains an uneven distribution of phototherapy clinics. As of 2015, there were 19 million individuals who qualified for Medicare without a clinic within driving distance.¹⁰ This problem likely also exists for many Medicaid patients who may not qualify for at-home UVB. In this scenario, tanning or heliotherapy may be effective alternatives.

In-Office vs At-Home Options—Although in-office UVB was the option considered safest (26.2%) and most efficacious (26.8%), it was followed closely by at-home UVB in both categories (safest, 23.8%; most efficacious, 24.2%). Meanwhile, at-home UVB (40.2%) was chosen as the most convenient. Some patients consider tanning options over in-office UVB because of the inconvenience of traveling to an appointment.¹¹ Therefore, at-home tanning may be a convenient alternative for these patients.

Considerations—Although our study was limited to an adult population, issues with convenience exist for the pediatric population as well, as children may need to miss multiple days of school each week to be treated in the office. For these pediatric patients, an at-home unit is preferable; however; issues with insurance coverage remain a challenge.¹² Increasing insurance coverage of at-home units for the pediatric population therefore would be most prudent. However, when other options have been exhausted, including in-office UVB, tanning and sunbathing may be viable alternatives because of cost and convenience. In our study, sunbathing (33.2%) was considered the most cost-effective, likely because it does not require expensive equipment or a visit to a salon or physician’s office. Sunbathing has been effective in treating some dermatologic conditions, such as atopic dermatitis.¹³ However, it may only be effective during certain months and at different latitudes—conditions that make UVB sun rays more accessible—particularly when treating psoriasis.¹⁴ Furthermore, sunbathing may not be as cost-effective in patients with average-severity psoriasis compared with conventional psoriasis therapy because of the costs of travel to areas with sufficient UVB rays for treatment.¹⁵ Additionally, insurance status was related to which option was selected as the most cost-effective, as 29 (53.7%) of 54 uninsured participants chose sunbathing as the most cost-effective option, while only 92 (34.2%) of 269 privately insured patients selected sunbathing. Therefore, insurance status may be a factor for dermatologists to consider if a patient prefers a treatment that is cost-effective. Overall, dermatologists could perhaps consider guiding patients and optimizing their treatment plans based on the factors most important to the patients while understanding that costs and insurance status may ultimately determine the treatment option.

Limitations—Survey participants were recruited on Amazon Mechanical Turk, which could create sampling bias. Furthermore, these participants were representative of the general public and not exclusively patients on phototherapy, therefore representing the opinions of the general public and not those who may require phototherapy. Furthermore, given the nature of the survey, the study was limited to the adult population.

Phototherapy—particularly UVB phototherapy, which utilizes UVB rays of specific wavelengths within the UV spectrum—is indicated for a wide variety of dermatoses. In-office and at-home UVB treatments commonly are used, as are salon tanning and sunbathing. When selecting a form of phototherapy, patients are likely to consider safety, cost, effectiveness, insurance issues, and convenience. Research on patient preferences; the reasons for these preferences; and which options patients perceive to be the safest, most cost-effective, efficacious, and convenient is lacking. We aimed to assess the forms of phototherapy that patients would most consider using; the factors influencing patient preferences; and the forms patients perceived as the safest and most cost-effective, efficacious, and convenient.

Methods

Study Participants—We recruited 500 Amazon Mechanical Turk users who were 18 years or older to complete our REDCap-generated survey. The study was approved by the Wake Forest University institutional review board (Winston-Salem, North Carolina).

Evaluation—Participants were asked, “If you were diagnosed with a skin disease that benefited from UV therapy, which of the following forms of UV therapy would you consider choosing?” Participants were instructed to choose all of the forms they would consider using. Available options included in-office UV, at-home UV, home tanning, salon tanning, sunbathing, and other. Participants were asked to select which factors—from safety, cost, effectiveness, issues with insurance, convenience, and other—influenced their decision-making; which form of phototherapy they would most consider along with the factors that influenced their preference for this specific form of phototherapy; and which options they considered to be safest and most cost-effective, efficacious, and convenient. Participants were asked to provide basic sociodemographic information, level of education, income, insurance status (private, Medicare, Medicaid, Veterans Affairs, and uninsured), and distance from the nearest dermatologist.

Statistical Analysis—Descriptive and inferential statistics (χ² test) were used to analyze the data with a significance set at P<.05.

Results

Five hundred participants completed the survey (Table 1).

Factors Influencing Patient Preferences—When asked to select all forms of phototherapy they would consider, 186 (37.2%) participants selected in-office UVB, 263 (52.6%) selected at-home UV, 141 (28.2%) selected home tanning, 117 (23.4%) selected salon tanning, 191 (38.2%) selected sunbathing, and 3 (0.6%) selected other. Participants who selected in-office UVB as an option were more likely to also select salon tanning (P<.012). No other relationship was found between the UVB options and the tanning options. When asked which factors influenced their phototherapy preferences, 295 (59%) selected convenience, 266 (53.2%) selected effectiveness, 220 (44%) selected safety, 218 (43.6%) selected cost, 72 (14.4%) selected issues with insurance, and 4 (0.8%) selected other. Forms of Phototherapy Patients Consider Using—When asked which form of phototherapy they would most consider using, 179 (35.8%) participants selected at-home UVB, 108 (21.6%) selected sunbathing, 92 (18.4%) selected in-office UVB, 62 (12.4%) selected home-tanning, 57 (11.4%) selected salon tanning, 1 (0.2%) selected other, and 1 participant provided no response (P<.001).

Reasons for Using Phototherapy—Of the 179 who selected at-home UVB, 125 (70%) cited convenience as a reason. Of the 108 who selected salon tanning as their top choice, 62 (57%) cited cost as a reason. Convenience (P<.001), cost (P<.001), and safety (P=.023) were related to top preference. Issues with insurance did not have a statistically significant relationship with the top preference. However, participant insurance type was related to top phototherapy preference (P=.021), with privately insured patients more likely to select in-office UVB, whereas those with Medicaid and Medicare were more likely to select home or salon tanning. Efficacy was not related to top preference. Furthermore, age, gender, education, income, and distance from nearest dermatologist were not related to top preference.

In-office UVB was perceived to be safest (P<.001) and most efficacious (P<.001). Meanwhile, at-home UVB was selected as most convenient (P<.001). Lastly, sunbathing was determined to be most cost-effective (P<.001)(Table 2). Cost-effectiveness had a relationship (P<.001) with the participant’s insurance, as those with private insurance were more likely to select at-home UVB, whereas those with Medicare or Medicaid were more likely to select the tanning options. Additionally, of the54 uninsured participants in the survey, 29 selected sunbathing as the most cost-effective option.

Comment

Phototherapy Treatment—UVB phototherapy at a wavelength of 290 to 320 nm (311–313 nm for narrowband UVB) is used to treat various dermatoses, including psoriasis and atopic dermatitis. UVB alters skin cytokines, induces apoptosis, promotes immunosuppression, causes DNA damage, and decreases the proliferation of dendritic cells and other cells of the innate immune system.¹ In-office and at-home UV therapies make use of UVB wavelengths for treatment, while tanning and sunbathing contain not only UVB but also potentially harmful UVA rays. The wavelengths for indoor tanning devices include UVB at 280 to 315 nm and UVA at 315 to 400 nm, which are similar to those of the sun but with a different ratio of UVB to UVA and more intense total UV.² When in-office and at-home UVB options are not available, various forms of tanning such as salon tanning and sunbathing may be alternatives that are widely used.³ One of the main reasons patients consider alternative phototherapy options is cost, as 1 in-office UVB treatment may cost $140, but a month of unlimited tanning may cost $30 or perhaps nothing if a patient has a gym membership with access to a tanning bed. Lack of insurance benefits covering phototherapy can exacerbate cost burden.⁴ However, tanning beds are associated with an increased risk for melanoma and nonmelanoma cancers.^5,6 Additionally, all forms of phototherapy are associated with photoaging, but it is more intense with tanning and heliotherapy because of the presence of UVA, which penetrates deeper into the dermis.⁷ Meanwhile, for those who choose UVB therapy, deciding between an in-office and at-home UVB treatment could be a matter of convenience, as patients must consider long trips to the physician’s office; insurance status, as some insurances may not cover at-home UVB; or efficacy, which might be influenced by the presence of a physician or other medical staff. In many cases, patients may not be informed that at-home UVB is an option.

Patient Preferences—At-home UVB therapy was the most popular option in our study population, with most participants (52.6%) considering using it, and 35.9% choosing it as their top choice over all other phototherapy options. Safety, cost, and convenience were all found to be related to the option participants would most consider using. Prior analysis between at-home UVB and in-office UVB for the treatment of psoriasis determined that at-home UVB is as safe and cost-effective as in-office UVB without the inconvenience of the patient having to take time out of the week to visit the physician’s office,^8,9 making at-home UVB an option dermatologists may strongly consider for patients who value safety, cost, and convenience. Oddly, efficacy was not related to the top preference, despite being the second highest–cited factor (53.2%) for which forms of phototherapy participants would consider using. For insurance coverage, those with Medicaid and Medicare selected the cheaper tanning options with higher-than-expected frequencies. Although problems with insurance were not related to the top preference, insurance status was related, suggesting that preferences are tied to cost. Of note, while the number of dermatologists that accept Medicare has increased in the last few years, there still remains an uneven distribution of phototherapy clinics. As of 2015, there were 19 million individuals who qualified for Medicare without a clinic within driving distance.¹⁰ This problem likely also exists for many Medicaid patients who may not qualify for at-home UVB. In this scenario, tanning or heliotherapy may be effective alternatives.

In-Office vs At-Home Options—Although in-office UVB was the option considered safest (26.2%) and most efficacious (26.8%), it was followed closely by at-home UVB in both categories (safest, 23.8%; most efficacious, 24.2%). Meanwhile, at-home UVB (40.2%) was chosen as the most convenient. Some patients consider tanning options over in-office UVB because of the inconvenience of traveling to an appointment.¹¹ Therefore, at-home tanning may be a convenient alternative for these patients.

Considerations—Although our study was limited to an adult population, issues with convenience exist for the pediatric population as well, as children may need to miss multiple days of school each week to be treated in the office. For these pediatric patients, an at-home unit is preferable; however; issues with insurance coverage remain a challenge.¹² Increasing insurance coverage of at-home units for the pediatric population therefore would be most prudent. However, when other options have been exhausted, including in-office UVB, tanning and sunbathing may be viable alternatives because of cost and convenience. In our study, sunbathing (33.2%) was considered the most cost-effective, likely because it does not require expensive equipment or a visit to a salon or physician’s office. Sunbathing has been effective in treating some dermatologic conditions, such as atopic dermatitis.¹³ However, it may only be effective during certain months and at different latitudes—conditions that make UVB sun rays more accessible—particularly when treating psoriasis.¹⁴ Furthermore, sunbathing may not be as cost-effective in patients with average-severity psoriasis compared with conventional psoriasis therapy because of the costs of travel to areas with sufficient UVB rays for treatment.¹⁵ Additionally, insurance status was related to which option was selected as the most cost-effective, as 29 (53.7%) of 54 uninsured participants chose sunbathing as the most cost-effective option, while only 92 (34.2%) of 269 privately insured patients selected sunbathing. Therefore, insurance status may be a factor for dermatologists to consider if a patient prefers a treatment that is cost-effective. Overall, dermatologists could perhaps consider guiding patients and optimizing their treatment plans based on the factors most important to the patients while understanding that costs and insurance status may ultimately determine the treatment option.

Limitations—Survey participants were recruited on Amazon Mechanical Turk, which could create sampling bias. Furthermore, these participants were representative of the general public and not exclusively patients on phototherapy, therefore representing the opinions of the general public and not those who may require phototherapy. Furthermore, given the nature of the survey, the study was limited to the adult population.

Phototherapy—particularly UVB phototherapy, which utilizes UVB rays of specific wavelengths within the UV spectrum—is indicated for a wide variety of dermatoses. In-office and at-home UVB treatments commonly are used, as are salon tanning and sunbathing. When selecting a form of phototherapy, patients are likely to consider safety, cost, effectiveness, insurance issues, and convenience. Research on patient preferences; the reasons for these preferences; and which options patients perceive to be the safest, most cost-effective, efficacious, and convenient is lacking. We aimed to assess the forms of phototherapy that patients would most consider using; the factors influencing patient preferences; and the forms patients perceived as the safest and most cost-effective, efficacious, and convenient.

Methods

Study Participants—We recruited 500 Amazon Mechanical Turk users who were 18 years or older to complete our REDCap-generated survey. The study was approved by the Wake Forest University institutional review board (Winston-Salem, North Carolina).

Evaluation—Participants were asked, “If you were diagnosed with a skin disease that benefited from UV therapy, which of the following forms of UV therapy would you consider choosing?” Participants were instructed to choose all of the forms they would consider using. Available options included in-office UV, at-home UV, home tanning, salon tanning, sunbathing, and other. Participants were asked to select which factors—from safety, cost, effectiveness, issues with insurance, convenience, and other—influenced their decision-making; which form of phototherapy they would most consider along with the factors that influenced their preference for this specific form of phototherapy; and which options they considered to be safest and most cost-effective, efficacious, and convenient. Participants were asked to provide basic sociodemographic information, level of education, income, insurance status (private, Medicare, Medicaid, Veterans Affairs, and uninsured), and distance from the nearest dermatologist.

Statistical Analysis—Descriptive and inferential statistics (χ² test) were used to analyze the data with a significance set at P<.05.

Results

Five hundred participants completed the survey (Table 1).

Factors Influencing Patient Preferences—When asked to select all forms of phototherapy they would consider, 186 (37.2%) participants selected in-office UVB, 263 (52.6%) selected at-home UV, 141 (28.2%) selected home tanning, 117 (23.4%) selected salon tanning, 191 (38.2%) selected sunbathing, and 3 (0.6%) selected other. Participants who selected in-office UVB as an option were more likely to also select salon tanning (P<.012). No other relationship was found between the UVB options and the tanning options. When asked which factors influenced their phototherapy preferences, 295 (59%) selected convenience, 266 (53.2%) selected effectiveness, 220 (44%) selected safety, 218 (43.6%) selected cost, 72 (14.4%) selected issues with insurance, and 4 (0.8%) selected other. Forms of Phototherapy Patients Consider Using—When asked which form of phototherapy they would most consider using, 179 (35.8%) participants selected at-home UVB, 108 (21.6%) selected sunbathing, 92 (18.4%) selected in-office UVB, 62 (12.4%) selected home-tanning, 57 (11.4%) selected salon tanning, 1 (0.2%) selected other, and 1 participant provided no response (P<.001).

Reasons for Using Phototherapy—Of the 179 who selected at-home UVB, 125 (70%) cited convenience as a reason. Of the 108 who selected salon tanning as their top choice, 62 (57%) cited cost as a reason. Convenience (P<.001), cost (P<.001), and safety (P=.023) were related to top preference. Issues with insurance did not have a statistically significant relationship with the top preference. However, participant insurance type was related to top phototherapy preference (P=.021), with privately insured patients more likely to select in-office UVB, whereas those with Medicaid and Medicare were more likely to select home or salon tanning. Efficacy was not related to top preference. Furthermore, age, gender, education, income, and distance from nearest dermatologist were not related to top preference.

In-office UVB was perceived to be safest (P<.001) and most efficacious (P<.001). Meanwhile, at-home UVB was selected as most convenient (P<.001). Lastly, sunbathing was determined to be most cost-effective (P<.001)(Table 2). Cost-effectiveness had a relationship (P<.001) with the participant’s insurance, as those with private insurance were more likely to select at-home UVB, whereas those with Medicare or Medicaid were more likely to select the tanning options. Additionally, of the54 uninsured participants in the survey, 29 selected sunbathing as the most cost-effective option.

Comment

Phototherapy Treatment—UVB phototherapy at a wavelength of 290 to 320 nm (311–313 nm for narrowband UVB) is used to treat various dermatoses, including psoriasis and atopic dermatitis. UVB alters skin cytokines, induces apoptosis, promotes immunosuppression, causes DNA damage, and decreases the proliferation of dendritic cells and other cells of the innate immune system.¹ In-office and at-home UV therapies make use of UVB wavelengths for treatment, while tanning and sunbathing contain not only UVB but also potentially harmful UVA rays. The wavelengths for indoor tanning devices include UVB at 280 to 315 nm and UVA at 315 to 400 nm, which are similar to those of the sun but with a different ratio of UVB to UVA and more intense total UV.² When in-office and at-home UVB options are not available, various forms of tanning such as salon tanning and sunbathing may be alternatives that are widely used.³ One of the main reasons patients consider alternative phototherapy options is cost, as 1 in-office UVB treatment may cost $140, but a month of unlimited tanning may cost $30 or perhaps nothing if a patient has a gym membership with access to a tanning bed. Lack of insurance benefits covering phototherapy can exacerbate cost burden.⁴ However, tanning beds are associated with an increased risk for melanoma and nonmelanoma cancers.^5,6 Additionally, all forms of phototherapy are associated with photoaging, but it is more intense with tanning and heliotherapy because of the presence of UVA, which penetrates deeper into the dermis.⁷ Meanwhile, for those who choose UVB therapy, deciding between an in-office and at-home UVB treatment could be a matter of convenience, as patients must consider long trips to the physician’s office; insurance status, as some insurances may not cover at-home UVB; or efficacy, which might be influenced by the presence of a physician or other medical staff. In many cases, patients may not be informed that at-home UVB is an option.

Patient Preferences—At-home UVB therapy was the most popular option in our study population, with most participants (52.6%) considering using it, and 35.9% choosing it as their top choice over all other phototherapy options. Safety, cost, and convenience were all found to be related to the option participants would most consider using. Prior analysis between at-home UVB and in-office UVB for the treatment of psoriasis determined that at-home UVB is as safe and cost-effective as in-office UVB without the inconvenience of the patient having to take time out of the week to visit the physician’s office,^8,9 making at-home UVB an option dermatologists may strongly consider for patients who value safety, cost, and convenience. Oddly, efficacy was not related to the top preference, despite being the second highest–cited factor (53.2%) for which forms of phototherapy participants would consider using. For insurance coverage, those with Medicaid and Medicare selected the cheaper tanning options with higher-than-expected frequencies. Although problems with insurance were not related to the top preference, insurance status was related, suggesting that preferences are tied to cost. Of note, while the number of dermatologists that accept Medicare has increased in the last few years, there still remains an uneven distribution of phototherapy clinics. As of 2015, there were 19 million individuals who qualified for Medicare without a clinic within driving distance.¹⁰ This problem likely also exists for many Medicaid patients who may not qualify for at-home UVB. In this scenario, tanning or heliotherapy may be effective alternatives.

In-Office vs At-Home Options—Although in-office UVB was the option considered safest (26.2%) and most efficacious (26.8%), it was followed closely by at-home UVB in both categories (safest, 23.8%; most efficacious, 24.2%). Meanwhile, at-home UVB (40.2%) was chosen as the most convenient. Some patients consider tanning options over in-office UVB because of the inconvenience of traveling to an appointment.¹¹ Therefore, at-home tanning may be a convenient alternative for these patients.

Considerations—Although our study was limited to an adult population, issues with convenience exist for the pediatric population as well, as children may need to miss multiple days of school each week to be treated in the office. For these pediatric patients, an at-home unit is preferable; however; issues with insurance coverage remain a challenge.¹² Increasing insurance coverage of at-home units for the pediatric population therefore would be most prudent. However, when other options have been exhausted, including in-office UVB, tanning and sunbathing may be viable alternatives because of cost and convenience. In our study, sunbathing (33.2%) was considered the most cost-effective, likely because it does not require expensive equipment or a visit to a salon or physician’s office. Sunbathing has been effective in treating some dermatologic conditions, such as atopic dermatitis.¹³ However, it may only be effective during certain months and at different latitudes—conditions that make UVB sun rays more accessible—particularly when treating psoriasis.¹⁴ Furthermore, sunbathing may not be as cost-effective in patients with average-severity psoriasis compared with conventional psoriasis therapy because of the costs of travel to areas with sufficient UVB rays for treatment.¹⁵ Additionally, insurance status was related to which option was selected as the most cost-effective, as 29 (53.7%) of 54 uninsured participants chose sunbathing as the most cost-effective option, while only 92 (34.2%) of 269 privately insured patients selected sunbathing. Therefore, insurance status may be a factor for dermatologists to consider if a patient prefers a treatment that is cost-effective. Overall, dermatologists could perhaps consider guiding patients and optimizing their treatment plans based on the factors most important to the patients while understanding that costs and insurance status may ultimately determine the treatment option.

Limitations—Survey participants were recruited on Amazon Mechanical Turk, which could create sampling bias. Furthermore, these participants were representative of the general public and not exclusively patients on phototherapy, therefore representing the opinions of the general public and not those who may require phototherapy. Furthermore, given the nature of the survey, the study was limited to the adult population.

Case Report

A 7-year-old girl who was otherwise healthy was referred by pediatric gastroenterology for evaluation of cutaneous Crohn disease (CD). The patient had a 4-year history of persistent lip swelling and a 3-year history of asymmetric erythematous labial swelling and perianal erythema with skin tags. She had been applying the calcineurin inhibitor tacrolimus ointment 0.03% 1 or 2 times daily to her lesions with minimal improvement. She did not have a medical history of recurrent or unusual infectious diseases. There was no family history of autoimmune disease.

The patient and her guardian reported intermittent perianal pain but denied constipation, diarrhea, abdominal pain, and blood in the stool. She denied throat and tongue swelling, dysphagia, dyspnea, drooling, facial paralysis, and eyelid edema. She was a well-nourished child whose height and weight percentiles tracked at 30% and 25%, respectively. Physical examination revealed confluent symmetric lip swelling with mild angular cheilitis. Multiple 1- to 2-mm white pustules with pinpoint erosions covered the upper and lower labial mucosa and extended onto the buccal mucosa (Figure 1). She had symmetric erythema and swelling of the left labia majora extending to and involving the left perianal mucosa. Three perianal erythematous skin tags and a perianal fissure were identified.

The patient had been assessed 2 years earlier by pediatric dermatology and gastroenterology with an extensive evaluation that favored a diagnosis of cutaneous CD because the combination of orofacial granulomatosis (OFG), vulvar edema, and perianal skin tags is strongly associated.^1-3 Contact dermatitis affecting the mouth was considered; however, allergen testing did not demonstrate a trigger.

A trial of a benzoate- and cinnamon-free diet, which has been reported to improve OFG,⁴ did not provide symptomatic improvement. Topical corticosteroids and tacrolimus reduced the perioral erythema, but the swelling persisted. An infectious cause was considered; however, topical mupirocin had no effect, and amoxicillin resulted in oral candidiasis.

A perianal biopsy revealed a granulomatous dermatitis. Fungal and bacterial cultures were negative. Upper and lower gastrointestinal (GI) endoscopy and a fecal calprotectin assay were not suggestive of inflammatory bowel disease (IBD). A complete blood cell count and QuantiFERON-TB Gold test measuring the immune response to tuberculosis antigens were normal. Chronic granulomatous disease, RAG1/RAG2 deficiency, common variable immunodeficiency, and NOD2 defects were ruled out with normal tests of dihydrorhodamine, quantitative immunoglobulins, and toll-like receptors.

Because of the discomfort associated with the patient’s lesions, she was offered treatment with tumor necrosis factor α inhibitors, including infliximab and adalimumab. These agents had been offered since the onset of symptoms; however, her parents declined systemic medication unless she developed GI involvement. Instead, the tacrolimus concentration was increased to 0.1% applied to the lips, labia, and perianal area, and fluocinonide gel 0.05% applied nightly to the oral pustules was added.

Two months later the patient had notably fewer oral pustules and diminished erythema but only slightly reduced oral, vulvar, and perianal swelling. A trial of oral metronidazole, which has been reported to clear a patient with cutaneous CD,⁵ was discontinued by her parents after 6 weeks because of a lack of interval improvement.

One year later, a pre-existing perianal skin tag doubled in size and became exquisitely tender. The calprotectin level—previously within reference range at less than 16 μg/g—was now elevated at 149 μg/g (reference range, 1–120 μg/g) and increased to 336 μg/g 3 weeks later. Testing for C-reactive protein, zinc, and stool occult blood; a comprehensive metabolic panel; and a complete blood cell count were unremarkable.

Repeat upper and lower GI endoscopy did not suggest CD. A biopsy using direct immunofluorescence (DIF) was obtained to evaluate for pyostomatitis vegetans (PSV) and rule out pemphigus vegetans of Hallopeau (PVH). Biopsy of a pustule was attempted but was challenging because of the patient’s age and difficulty cooperating.

The captured biopsy did not demonstrate the intended pustule; instead, it included less-affected mucosa and was obtained during topical treatment when few pustules and erosions persisted. Pathologic analysis revealed noncaseating granulomas without an increase in microabscesses, neutrophils, or eosinophils (Figure 2). Direct immunofluorescence staining for IgG, IgA, and C3 and indirect immunofluorescence staining for desmoglein-1 and desmoglein-3 antibodies were negative. Although the biopsy did not capture the intended pustule, diagnosis of PV was made based on clinical features and the constellation of cutaneous findings associated with IBD.

Intralesional triamcinolone, which has been of benefit for pediatric patients with orofacial granulomatosis,^1,6,7 was instituted and normalized the vulva and perianal mucosa; however, lip swelling improved only minimally.

Comment

Pyostomatitis vegetans is characterized by multiple white or yellow, friable, miliary pustules that rupture, leaving behind ulcerations and erosions that cause a varying degree of oral pain.⁸ The disorder can involve any area of the oral mucosa—most often the labia-attached gingiva, soft and hard palates, buccal mucosa, vestibule, and tonsillar areas—but often spares the floor of the mouth and tongue.^8-11 The term pyostomatitis vegetans was proposed in 1949 by McCarthy¹² when he noted in a patient who presented with the characteristic appearance of the oral mucosa, though cases of vaginal, nasal, and periocular involvement have been reported.^8,13,14

Histopathology—Pyostomatitis vegetans displays pseudoepithelial hyperplasia with acanthosis, hyperkeratosis, and intraepithelial or subepithelial microabscesses (or both) with neutrophils and eosinophils.^8,9,15 There are a few possible explanations for this patient’s lack of tissue eosinophilia. It has been theorized that the presence of granulomas could mask concurrent PSV¹⁶ or that tissue in PSV contains fewer eosinophils as the disorder progresses.¹¹ The oral biopsy obtained from our patient did not capture a pustule, and the condition had noticeably improved with topical tacrolimus at the time of biopsy; therefore, neither neutrophils nor eosinophils were identified. Peripheral eosinophilia, which is present in 42% to 90% of cases of PSV,^9,17 can be a diagnostic clue.¹⁸ However, PE is associated with IBD,²⁴ which usually occurs with PSV, so the absence of peripheral eosinophilia in our patient may be explained by her lack of bowel disease.

Pathogenesis—The pathogenesis of PSV is unknown. A proposed etiology includes cross-reacting antigens in the bowel and skin secondary to IBD as well as an aberrant immune response to an unidentified factor.⁸ Pyostomatitis vegetans is considered by many to be the mucosal variant of pyodermatitis vegetans,^9,15,19 a neutrophilic dermatosis characterized by asymmetric, crusted, erythematous papulopustules that extend peripherally and coalesce to form large vegetating plaques. These lesions commonly manifest in the axillary folds, groin, and scalp and can involve the face, trunk, and distal extremities.^9,18 Infection has been suggested as a cause of PSV, though cultures for pathogenic bacteria, viruses, and fungi consistently show only normal flora.²⁰ Zinc deficiency attributed to malabsorption from CD was reported in an adult with PSV.²¹ The PSV resolved after 6 weeks of zinc supplementation.

Differential Diagnosis—The main entity in the clinical differential diagnosis for PSV is PVH, which is considered a variant of pemphigus vulgaris. Pemphigus vegetans of Hallopeau presents with pustules and progresses to hyperpigmented vegetative plaques with peripheral hypertrophic granulation tissue.²² The clinical and histological presentation of PVH can be similar to PSV; in PVH, however, DIF demonstrates intercellular IgG and C3 due to circulating IgG autoantibodies specific for desmoglein 3, a cell adhesion molecule.^22-24 In PSV, DIF typically is negative for IgG, IgA, and C3.⁸ Immunohistochemical findings of PSV may overlap with IgA pemphigus, IgG/IgA pemphigus, and IgG pemphigus, which has sparked debate if PSV is an autoimmune blistering disorder or a secondary finding of epithelial injury.^9,18,24

Pyostomatitis vegetans is most prevalent in patients aged 20 to 59 years²⁵ but can occur at any age.^8,19 Overall, extraintestinal symptoms, including mucocutaneous findings, are common in pediatric patients—in 30% to 71% of children with CD and 21% to 22% of children with ulcerative colitis²⁶—and can predate onset of GI symptoms in 6% of pediatric patients.²⁷

Oral disease is common in CD; manifestations are listed in the Table.^28,29 In a prospective study of 48 children with CD, 42% (20/48) had oral manifestations identified at diagnosis²⁸; in a similar study of 25 children, researchers noted that 48% (12/25) had disease-specific oral lesions.²⁹ None of these children recognized the oral findings prior to the onset of systemic symptoms.²⁸ Pyostomatitis vegetans was the least common oral manifestation, reported in 1 of 73 patients in the 2 studies combined.^28,29

Two recent articles that looked at PSV in pediatric and adolescent populations identified only 9 patients with PSV.^24,30 Only 2 patients (siblings) had documented onset of PSV before 12 years of age,³¹ which suggests an underlying genetic predisposition in young children.

It has been reported that active or subclinical (ie, asymptomatic with positive endoscopic findings) IBD in adults precedes onset of PSV, which may be considered a sign of relapse.^9,30 However, PSV is incredibly rare in children and adolescents and can be an early finding of IBD in children.^16,31,32

Our patient has not developed GI involvement since her initial presentation 5 years prior, though another pediatric patient developed symptomatic CD 9 years after onset of OFG.⁵ A retrospective review of pediatric OFG without CD met criteria for CD at a median of 3.1 years (range, 0.4–6.9 years).³³ Regrettably, the early presence of PSV has been associated with future progression to CD and a complicated disease course.^12,34

Management—Pyoderma stomatitis vegetans is treated with management of underlying IBD,⁸ with scarce literature available regarding pediatric patients. Oral lesions have been treated with antiseptics and topical corticosteroids, though these have limited benefit.⁸ In an adult with IBD, topical tacrolimus initially cleared PSV; however, lesions recurred until mesalamine was initiated.³⁵ Systemic steroids were effective in a 16-year-old patient with CD and PSV,¹² but recurrence is common after corticosteroids are stopped.³⁴

Some patients benefit from steroid-sparing medications, such as dapsone, azathioprine, sulfamethoxypyridazine, methotrexate, mycophenolate mofetil, and tumor necrosis factor α inhibitors such as infliximab and adalimumab.^{8,9,15,23,34,36} A 12-year-old patient with pyodermatitis–PSV without intestinal disease was treated with prednisone, dapsone, and azathioprine with improvement but not complete resolution of oral erosions after 18 weeks of treatment.³² A 15-year-old patient with CD and pyodermatitis–PSV did not show improvement on prednisone, dapsone, and azathioprine but rapidly responded to infliximab.²³ Infliximab led to complete clearance of oral lesions in an adult with severe fistulizing CD who developed PSV.¹¹ However, 2 adolescent patients with CD developed PSV while on adalimumab,^6,34 though 1 did improve after increasing adalimumab from once to twice weekly.⁶

Conclusion

The case described here—PSV in a prepubertal 7-year-old with multiple cutaneous findings suggestive of CD, including OFG, perianal and vulvar edema with biopsy-proven noncaseating granulomas, anal skin tags, and an elevated calprotectin level, noted during a cutaneous flare without clinical or endoscopically identified underlying bowel involvement—is an extremely rare presentation. Literature regarding management of PSV primarily is found in the form of case reports and focuses on treating underlying IBD. In patients with intestinal disease, treatment with biologic therapy appears most effective.^6,23

ADDENDUM

Interestingly, 3 years after the patient’s original presentation to our clinic, chromosomal sequencing analysis to assess for copy number variants and whole exome gene sequencing identified a variant of unknown significance in the heat shock protein family A member 1-like gene, HSPA1L, which has an unknown mode of inheritance, but the literature suggests that both truncating and missense variants could be associated with individuals with ulcerative colitis, CD, and IBD.^37,38 Although we cannot use this information to render a molecular diagnosis, it is highly suspicious that this is the cause of her clinical findings. Additionally, the patient currently is aged 10 years with unchanged cutaneous findings and has not developed gastrointestinal findings of IBD.

Case Report

A 7-year-old girl who was otherwise healthy was referred by pediatric gastroenterology for evaluation of cutaneous Crohn disease (CD). The patient had a 4-year history of persistent lip swelling and a 3-year history of asymmetric erythematous labial swelling and perianal erythema with skin tags. She had been applying the calcineurin inhibitor tacrolimus ointment 0.03% 1 or 2 times daily to her lesions with minimal improvement. She did not have a medical history of recurrent or unusual infectious diseases. There was no family history of autoimmune disease.

The patient and her guardian reported intermittent perianal pain but denied constipation, diarrhea, abdominal pain, and blood in the stool. She denied throat and tongue swelling, dysphagia, dyspnea, drooling, facial paralysis, and eyelid edema. She was a well-nourished child whose height and weight percentiles tracked at 30% and 25%, respectively. Physical examination revealed confluent symmetric lip swelling with mild angular cheilitis. Multiple 1- to 2-mm white pustules with pinpoint erosions covered the upper and lower labial mucosa and extended onto the buccal mucosa (Figure 1). She had symmetric erythema and swelling of the left labia majora extending to and involving the left perianal mucosa. Three perianal erythematous skin tags and a perianal fissure were identified.

The patient had been assessed 2 years earlier by pediatric dermatology and gastroenterology with an extensive evaluation that favored a diagnosis of cutaneous CD because the combination of orofacial granulomatosis (OFG), vulvar edema, and perianal skin tags is strongly associated.^1-3 Contact dermatitis affecting the mouth was considered; however, allergen testing did not demonstrate a trigger.

A trial of a benzoate- and cinnamon-free diet, which has been reported to improve OFG,⁴ did not provide symptomatic improvement. Topical corticosteroids and tacrolimus reduced the perioral erythema, but the swelling persisted. An infectious cause was considered; however, topical mupirocin had no effect, and amoxicillin resulted in oral candidiasis.

A perianal biopsy revealed a granulomatous dermatitis. Fungal and bacterial cultures were negative. Upper and lower gastrointestinal (GI) endoscopy and a fecal calprotectin assay were not suggestive of inflammatory bowel disease (IBD). A complete blood cell count and QuantiFERON-TB Gold test measuring the immune response to tuberculosis antigens were normal. Chronic granulomatous disease, RAG1/RAG2 deficiency, common variable immunodeficiency, and NOD2 defects were ruled out with normal tests of dihydrorhodamine, quantitative immunoglobulins, and toll-like receptors.

Because of the discomfort associated with the patient’s lesions, she was offered treatment with tumor necrosis factor α inhibitors, including infliximab and adalimumab. These agents had been offered since the onset of symptoms; however, her parents declined systemic medication unless she developed GI involvement. Instead, the tacrolimus concentration was increased to 0.1% applied to the lips, labia, and perianal area, and fluocinonide gel 0.05% applied nightly to the oral pustules was added.

Two months later the patient had notably fewer oral pustules and diminished erythema but only slightly reduced oral, vulvar, and perianal swelling. A trial of oral metronidazole, which has been reported to clear a patient with cutaneous CD,⁵ was discontinued by her parents after 6 weeks because of a lack of interval improvement.

One year later, a pre-existing perianal skin tag doubled in size and became exquisitely tender. The calprotectin level—previously within reference range at less than 16 μg/g—was now elevated at 149 μg/g (reference range, 1–120 μg/g) and increased to 336 μg/g 3 weeks later. Testing for C-reactive protein, zinc, and stool occult blood; a comprehensive metabolic panel; and a complete blood cell count were unremarkable.

Repeat upper and lower GI endoscopy did not suggest CD. A biopsy using direct immunofluorescence (DIF) was obtained to evaluate for pyostomatitis vegetans (PSV) and rule out pemphigus vegetans of Hallopeau (PVH). Biopsy of a pustule was attempted but was challenging because of the patient’s age and difficulty cooperating.

The captured biopsy did not demonstrate the intended pustule; instead, it included less-affected mucosa and was obtained during topical treatment when few pustules and erosions persisted. Pathologic analysis revealed noncaseating granulomas without an increase in microabscesses, neutrophils, or eosinophils (Figure 2). Direct immunofluorescence staining for IgG, IgA, and C3 and indirect immunofluorescence staining for desmoglein-1 and desmoglein-3 antibodies were negative. Although the biopsy did not capture the intended pustule, diagnosis of PV was made based on clinical features and the constellation of cutaneous findings associated with IBD.

Intralesional triamcinolone, which has been of benefit for pediatric patients with orofacial granulomatosis,^1,6,7 was instituted and normalized the vulva and perianal mucosa; however, lip swelling improved only minimally.

Comment

Pyostomatitis vegetans is characterized by multiple white or yellow, friable, miliary pustules that rupture, leaving behind ulcerations and erosions that cause a varying degree of oral pain.⁸ The disorder can involve any area of the oral mucosa—most often the labia-attached gingiva, soft and hard palates, buccal mucosa, vestibule, and tonsillar areas—but often spares the floor of the mouth and tongue.^8-11 The term pyostomatitis vegetans was proposed in 1949 by McCarthy¹² when he noted in a patient who presented with the characteristic appearance of the oral mucosa, though cases of vaginal, nasal, and periocular involvement have been reported.^8,13,14

Histopathology—Pyostomatitis vegetans displays pseudoepithelial hyperplasia with acanthosis, hyperkeratosis, and intraepithelial or subepithelial microabscesses (or both) with neutrophils and eosinophils.^8,9,15 There are a few possible explanations for this patient’s lack of tissue eosinophilia. It has been theorized that the presence of granulomas could mask concurrent PSV¹⁶ or that tissue in PSV contains fewer eosinophils as the disorder progresses.¹¹ The oral biopsy obtained from our patient did not capture a pustule, and the condition had noticeably improved with topical tacrolimus at the time of biopsy; therefore, neither neutrophils nor eosinophils were identified. Peripheral eosinophilia, which is present in 42% to 90% of cases of PSV,^9,17 can be a diagnostic clue.¹⁸ However, PE is associated with IBD,²⁴ which usually occurs with PSV, so the absence of peripheral eosinophilia in our patient may be explained by her lack of bowel disease.

Pathogenesis—The pathogenesis of PSV is unknown. A proposed etiology includes cross-reacting antigens in the bowel and skin secondary to IBD as well as an aberrant immune response to an unidentified factor.⁸ Pyostomatitis vegetans is considered by many to be the mucosal variant of pyodermatitis vegetans,^9,15,19 a neutrophilic dermatosis characterized by asymmetric, crusted, erythematous papulopustules that extend peripherally and coalesce to form large vegetating plaques. These lesions commonly manifest in the axillary folds, groin, and scalp and can involve the face, trunk, and distal extremities.^9,18 Infection has been suggested as a cause of PSV, though cultures for pathogenic bacteria, viruses, and fungi consistently show only normal flora.²⁰ Zinc deficiency attributed to malabsorption from CD was reported in an adult with PSV.²¹ The PSV resolved after 6 weeks of zinc supplementation.

Differential Diagnosis—The main entity in the clinical differential diagnosis for PSV is PVH, which is considered a variant of pemphigus vulgaris. Pemphigus vegetans of Hallopeau presents with pustules and progresses to hyperpigmented vegetative plaques with peripheral hypertrophic granulation tissue.²² The clinical and histological presentation of PVH can be similar to PSV; in PVH, however, DIF demonstrates intercellular IgG and C3 due to circulating IgG autoantibodies specific for desmoglein 3, a cell adhesion molecule.^22-24 In PSV, DIF typically is negative for IgG, IgA, and C3.⁸ Immunohistochemical findings of PSV may overlap with IgA pemphigus, IgG/IgA pemphigus, and IgG pemphigus, which has sparked debate if PSV is an autoimmune blistering disorder or a secondary finding of epithelial injury.^9,18,24

Pyostomatitis vegetans is most prevalent in patients aged 20 to 59 years²⁵ but can occur at any age.^8,19 Overall, extraintestinal symptoms, including mucocutaneous findings, are common in pediatric patients—in 30% to 71% of children with CD and 21% to 22% of children with ulcerative colitis²⁶—and can predate onset of GI symptoms in 6% of pediatric patients.²⁷

Oral disease is common in CD; manifestations are listed in the Table.^28,29 In a prospective study of 48 children with CD, 42% (20/48) had oral manifestations identified at diagnosis²⁸; in a similar study of 25 children, researchers noted that 48% (12/25) had disease-specific oral lesions.²⁹ None of these children recognized the oral findings prior to the onset of systemic symptoms.²⁸ Pyostomatitis vegetans was the least common oral manifestation, reported in 1 of 73 patients in the 2 studies combined.^28,29

Two recent articles that looked at PSV in pediatric and adolescent populations identified only 9 patients with PSV.^24,30 Only 2 patients (siblings) had documented onset of PSV before 12 years of age,³¹ which suggests an underlying genetic predisposition in young children.

It has been reported that active or subclinical (ie, asymptomatic with positive endoscopic findings) IBD in adults precedes onset of PSV, which may be considered a sign of relapse.^9,30 However, PSV is incredibly rare in children and adolescents and can be an early finding of IBD in children.^16,31,32

Our patient has not developed GI involvement since her initial presentation 5 years prior, though another pediatric patient developed symptomatic CD 9 years after onset of OFG.⁵ A retrospective review of pediatric OFG without CD met criteria for CD at a median of 3.1 years (range, 0.4–6.9 years).³³ Regrettably, the early presence of PSV has been associated with future progression to CD and a complicated disease course.^12,34

Management—Pyoderma stomatitis vegetans is treated with management of underlying IBD,⁸ with scarce literature available regarding pediatric patients. Oral lesions have been treated with antiseptics and topical corticosteroids, though these have limited benefit.⁸ In an adult with IBD, topical tacrolimus initially cleared PSV; however, lesions recurred until mesalamine was initiated.³⁵ Systemic steroids were effective in a 16-year-old patient with CD and PSV,¹² but recurrence is common after corticosteroids are stopped.³⁴

Some patients benefit from steroid-sparing medications, such as dapsone, azathioprine, sulfamethoxypyridazine, methotrexate, mycophenolate mofetil, and tumor necrosis factor α inhibitors such as infliximab and adalimumab.^{8,9,15,23,34,36} A 12-year-old patient with pyodermatitis–PSV without intestinal disease was treated with prednisone, dapsone, and azathioprine with improvement but not complete resolution of oral erosions after 18 weeks of treatment.³² A 15-year-old patient with CD and pyodermatitis–PSV did not show improvement on prednisone, dapsone, and azathioprine but rapidly responded to infliximab.²³ Infliximab led to complete clearance of oral lesions in an adult with severe fistulizing CD who developed PSV.¹¹ However, 2 adolescent patients with CD developed PSV while on adalimumab,^6,34 though 1 did improve after increasing adalimumab from once to twice weekly.⁶

Conclusion

The case described here—PSV in a prepubertal 7-year-old with multiple cutaneous findings suggestive of CD, including OFG, perianal and vulvar edema with biopsy-proven noncaseating granulomas, anal skin tags, and an elevated calprotectin level, noted during a cutaneous flare without clinical or endoscopically identified underlying bowel involvement—is an extremely rare presentation. Literature regarding management of PSV primarily is found in the form of case reports and focuses on treating underlying IBD. In patients with intestinal disease, treatment with biologic therapy appears most effective.^6,23

ADDENDUM

Interestingly, 3 years after the patient’s original presentation to our clinic, chromosomal sequencing analysis to assess for copy number variants and whole exome gene sequencing identified a variant of unknown significance in the heat shock protein family A member 1-like gene, HSPA1L, which has an unknown mode of inheritance, but the literature suggests that both truncating and missense variants could be associated with individuals with ulcerative colitis, CD, and IBD.^37,38 Although we cannot use this information to render a molecular diagnosis, it is highly suspicious that this is the cause of her clinical findings. Additionally, the patient currently is aged 10 years with unchanged cutaneous findings and has not developed gastrointestinal findings of IBD.

Case Report

A 7-year-old girl who was otherwise healthy was referred by pediatric gastroenterology for evaluation of cutaneous Crohn disease (CD). The patient had a 4-year history of persistent lip swelling and a 3-year history of asymmetric erythematous labial swelling and perianal erythema with skin tags. She had been applying the calcineurin inhibitor tacrolimus ointment 0.03% 1 or 2 times daily to her lesions with minimal improvement. She did not have a medical history of recurrent or unusual infectious diseases. There was no family history of autoimmune disease.

The patient and her guardian reported intermittent perianal pain but denied constipation, diarrhea, abdominal pain, and blood in the stool. She denied throat and tongue swelling, dysphagia, dyspnea, drooling, facial paralysis, and eyelid edema. She was a well-nourished child whose height and weight percentiles tracked at 30% and 25%, respectively. Physical examination revealed confluent symmetric lip swelling with mild angular cheilitis. Multiple 1- to 2-mm white pustules with pinpoint erosions covered the upper and lower labial mucosa and extended onto the buccal mucosa (Figure 1). She had symmetric erythema and swelling of the left labia majora extending to and involving the left perianal mucosa. Three perianal erythematous skin tags and a perianal fissure were identified.

The patient had been assessed 2 years earlier by pediatric dermatology and gastroenterology with an extensive evaluation that favored a diagnosis of cutaneous CD because the combination of orofacial granulomatosis (OFG), vulvar edema, and perianal skin tags is strongly associated.^1-3 Contact dermatitis affecting the mouth was considered; however, allergen testing did not demonstrate a trigger.

A trial of a benzoate- and cinnamon-free diet, which has been reported to improve OFG,⁴ did not provide symptomatic improvement. Topical corticosteroids and tacrolimus reduced the perioral erythema, but the swelling persisted. An infectious cause was considered; however, topical mupirocin had no effect, and amoxicillin resulted in oral candidiasis.

A perianal biopsy revealed a granulomatous dermatitis. Fungal and bacterial cultures were negative. Upper and lower gastrointestinal (GI) endoscopy and a fecal calprotectin assay were not suggestive of inflammatory bowel disease (IBD). A complete blood cell count and QuantiFERON-TB Gold test measuring the immune response to tuberculosis antigens were normal. Chronic granulomatous disease, RAG1/RAG2 deficiency, common variable immunodeficiency, and NOD2 defects were ruled out with normal tests of dihydrorhodamine, quantitative immunoglobulins, and toll-like receptors.

Because of the discomfort associated with the patient’s lesions, she was offered treatment with tumor necrosis factor α inhibitors, including infliximab and adalimumab. These agents had been offered since the onset of symptoms; however, her parents declined systemic medication unless she developed GI involvement. Instead, the tacrolimus concentration was increased to 0.1% applied to the lips, labia, and perianal area, and fluocinonide gel 0.05% applied nightly to the oral pustules was added.

Two months later the patient had notably fewer oral pustules and diminished erythema but only slightly reduced oral, vulvar, and perianal swelling. A trial of oral metronidazole, which has been reported to clear a patient with cutaneous CD,⁵ was discontinued by her parents after 6 weeks because of a lack of interval improvement.

One year later, a pre-existing perianal skin tag doubled in size and became exquisitely tender. The calprotectin level—previously within reference range at less than 16 μg/g—was now elevated at 149 μg/g (reference range, 1–120 μg/g) and increased to 336 μg/g 3 weeks later. Testing for C-reactive protein, zinc, and stool occult blood; a comprehensive metabolic panel; and a complete blood cell count were unremarkable.

Repeat upper and lower GI endoscopy did not suggest CD. A biopsy using direct immunofluorescence (DIF) was obtained to evaluate for pyostomatitis vegetans (PSV) and rule out pemphigus vegetans of Hallopeau (PVH). Biopsy of a pustule was attempted but was challenging because of the patient’s age and difficulty cooperating.

The captured biopsy did not demonstrate the intended pustule; instead, it included less-affected mucosa and was obtained during topical treatment when few pustules and erosions persisted. Pathologic analysis revealed noncaseating granulomas without an increase in microabscesses, neutrophils, or eosinophils (Figure 2). Direct immunofluorescence staining for IgG, IgA, and C3 and indirect immunofluorescence staining for desmoglein-1 and desmoglein-3 antibodies were negative. Although the biopsy did not capture the intended pustule, diagnosis of PV was made based on clinical features and the constellation of cutaneous findings associated with IBD.

Intralesional triamcinolone, which has been of benefit for pediatric patients with orofacial granulomatosis,^1,6,7 was instituted and normalized the vulva and perianal mucosa; however, lip swelling improved only minimally.

Comment

Pyostomatitis vegetans is characterized by multiple white or yellow, friable, miliary pustules that rupture, leaving behind ulcerations and erosions that cause a varying degree of oral pain.⁸ The disorder can involve any area of the oral mucosa—most often the labia-attached gingiva, soft and hard palates, buccal mucosa, vestibule, and tonsillar areas—but often spares the floor of the mouth and tongue.^8-11 The term pyostomatitis vegetans was proposed in 1949 by McCarthy¹² when he noted in a patient who presented with the characteristic appearance of the oral mucosa, though cases of vaginal, nasal, and periocular involvement have been reported.^8,13,14

Histopathology—Pyostomatitis vegetans displays pseudoepithelial hyperplasia with acanthosis, hyperkeratosis, and intraepithelial or subepithelial microabscesses (or both) with neutrophils and eosinophils.^8,9,15 There are a few possible explanations for this patient’s lack of tissue eosinophilia. It has been theorized that the presence of granulomas could mask concurrent PSV¹⁶ or that tissue in PSV contains fewer eosinophils as the disorder progresses.¹¹ The oral biopsy obtained from our patient did not capture a pustule, and the condition had noticeably improved with topical tacrolimus at the time of biopsy; therefore, neither neutrophils nor eosinophils were identified. Peripheral eosinophilia, which is present in 42% to 90% of cases of PSV,^9,17 can be a diagnostic clue.¹⁸ However, PE is associated with IBD,²⁴ which usually occurs with PSV, so the absence of peripheral eosinophilia in our patient may be explained by her lack of bowel disease.

Pathogenesis—The pathogenesis of PSV is unknown. A proposed etiology includes cross-reacting antigens in the bowel and skin secondary to IBD as well as an aberrant immune response to an unidentified factor.⁸ Pyostomatitis vegetans is considered by many to be the mucosal variant of pyodermatitis vegetans,^9,15,19 a neutrophilic dermatosis characterized by asymmetric, crusted, erythematous papulopustules that extend peripherally and coalesce to form large vegetating plaques. These lesions commonly manifest in the axillary folds, groin, and scalp and can involve the face, trunk, and distal extremities.^9,18 Infection has been suggested as a cause of PSV, though cultures for pathogenic bacteria, viruses, and fungi consistently show only normal flora.²⁰ Zinc deficiency attributed to malabsorption from CD was reported in an adult with PSV.²¹ The PSV resolved after 6 weeks of zinc supplementation.

Differential Diagnosis—The main entity in the clinical differential diagnosis for PSV is PVH, which is considered a variant of pemphigus vulgaris. Pemphigus vegetans of Hallopeau presents with pustules and progresses to hyperpigmented vegetative plaques with peripheral hypertrophic granulation tissue.²² The clinical and histological presentation of PVH can be similar to PSV; in PVH, however, DIF demonstrates intercellular IgG and C3 due to circulating IgG autoantibodies specific for desmoglein 3, a cell adhesion molecule.^22-24 In PSV, DIF typically is negative for IgG, IgA, and C3.⁸ Immunohistochemical findings of PSV may overlap with IgA pemphigus, IgG/IgA pemphigus, and IgG pemphigus, which has sparked debate if PSV is an autoimmune blistering disorder or a secondary finding of epithelial injury.^9,18,24

Pyostomatitis vegetans is most prevalent in patients aged 20 to 59 years²⁵ but can occur at any age.^8,19 Overall, extraintestinal symptoms, including mucocutaneous findings, are common in pediatric patients—in 30% to 71% of children with CD and 21% to 22% of children with ulcerative colitis²⁶—and can predate onset of GI symptoms in 6% of pediatric patients.²⁷

Oral disease is common in CD; manifestations are listed in the Table.^28,29 In a prospective study of 48 children with CD, 42% (20/48) had oral manifestations identified at diagnosis²⁸; in a similar study of 25 children, researchers noted that 48% (12/25) had disease-specific oral lesions.²⁹ None of these children recognized the oral findings prior to the onset of systemic symptoms.²⁸ Pyostomatitis vegetans was the least common oral manifestation, reported in 1 of 73 patients in the 2 studies combined.^28,29

Two recent articles that looked at PSV in pediatric and adolescent populations identified only 9 patients with PSV.^24,30 Only 2 patients (siblings) had documented onset of PSV before 12 years of age,³¹ which suggests an underlying genetic predisposition in young children.

It has been reported that active or subclinical (ie, asymptomatic with positive endoscopic findings) IBD in adults precedes onset of PSV, which may be considered a sign of relapse.^9,30 However, PSV is incredibly rare in children and adolescents and can be an early finding of IBD in children.^16,31,32

Our patient has not developed GI involvement since her initial presentation 5 years prior, though another pediatric patient developed symptomatic CD 9 years after onset of OFG.⁵ A retrospective review of pediatric OFG without CD met criteria for CD at a median of 3.1 years (range, 0.4–6.9 years).³³ Regrettably, the early presence of PSV has been associated with future progression to CD and a complicated disease course.^12,34

Management—Pyoderma stomatitis vegetans is treated with management of underlying IBD,⁸ with scarce literature available regarding pediatric patients. Oral lesions have been treated with antiseptics and topical corticosteroids, though these have limited benefit.⁸ In an adult with IBD, topical tacrolimus initially cleared PSV; however, lesions recurred until mesalamine was initiated.³⁵ Systemic steroids were effective in a 16-year-old patient with CD and PSV,¹² but recurrence is common after corticosteroids are stopped.³⁴

Some patients benefit from steroid-sparing medications, such as dapsone, azathioprine, sulfamethoxypyridazine, methotrexate, mycophenolate mofetil, and tumor necrosis factor α inhibitors such as infliximab and adalimumab.^{8,9,15,23,34,36} A 12-year-old patient with pyodermatitis–PSV without intestinal disease was treated with prednisone, dapsone, and azathioprine with improvement but not complete resolution of oral erosions after 18 weeks of treatment.³² A 15-year-old patient with CD and pyodermatitis–PSV did not show improvement on prednisone, dapsone, and azathioprine but rapidly responded to infliximab.²³ Infliximab led to complete clearance of oral lesions in an adult with severe fistulizing CD who developed PSV.¹¹ However, 2 adolescent patients with CD developed PSV while on adalimumab,^6,34 though 1 did improve after increasing adalimumab from once to twice weekly.⁶

Conclusion

The case described here—PSV in a prepubertal 7-year-old with multiple cutaneous findings suggestive of CD, including OFG, perianal and vulvar edema with biopsy-proven noncaseating granulomas, anal skin tags, and an elevated calprotectin level, noted during a cutaneous flare without clinical or endoscopically identified underlying bowel involvement—is an extremely rare presentation. Literature regarding management of PSV primarily is found in the form of case reports and focuses on treating underlying IBD. In patients with intestinal disease, treatment with biologic therapy appears most effective.^6,23

ADDENDUM

Interestingly, 3 years after the patient’s original presentation to our clinic, chromosomal sequencing analysis to assess for copy number variants and whole exome gene sequencing identified a variant of unknown significance in the heat shock protein family A member 1-like gene, HSPA1L, which has an unknown mode of inheritance, but the literature suggests that both truncating and missense variants could be associated with individuals with ulcerative colitis, CD, and IBD.^37,38 Although we cannot use this information to render a molecular diagnosis, it is highly suspicious that this is the cause of her clinical findings. Additionally, the patient currently is aged 10 years with unchanged cutaneous findings and has not developed gastrointestinal findings of IBD.

The percentage of people in the U.S. getting the latest COVID-19 booster shot has crept up by single digits in the past couple months, despite health officials pleading for people to do so before the Christmas holiday. 

Now, a new poll shows why so few people are willing to roll up their sleeves again.

The most common reasons people give for not getting the latest booster shot is that they “don’t think they need one” (44%) and they “don’t think the benefits are worth it” (37%), according to poll results from the Kaiser Family Foundation. 

The data comes amid announcements by the Centers for Disease Control and Prevention that boosters reduced COVID-19 hospitalizations by up to 57% for U.S. adults and by up to 84% for people age 65 and older. Those figures are just the latest in a mountain of research reporting the public health benefits of COVID-19 vaccines.

Despite all of the statistical data, health officials’ recent vaccination campaigns have proven far from compelling. 

So far, just 15% of people age 12 and older have gotten the latest booster, and 36% of people age 65 and older have gotten it, the CDC’s vaccination trackershows.

Since the start of the pandemic, 1.1 million people in the U.S. have died from COVID-19, with the number of deaths currently rising by 400 per day, The New York Times COVID tracker shows.

Many experts continue to note the need for everyone to get booster shots regularly, but some advocate that perhaps a change in strategy is in order.

“What the administration should do is push for vaccinating people in high-risk groups, including those who are older, those who are immunocompromised and those who have comorbidities,” Paul Offitt, MD, director of the Vaccine Education Center at Children’s Hospital of Philadelphia, told CNN.

Federal regulators have announced they will meet Jan. 26 with a panel of vaccine advisors to examine the current recommended vaccination schedule as well as look at the effectiveness and composition of current vaccines and boosters, with an eye toward the make-up of next-generation shots.

Vaccines are the “best available protection” against hospitalization and death caused by COVID-19, said Peter Marks, MD, PhD, director of the FDA’s Center for Biologics Evaluation and Research, in a statement announcing the planned meeting.

“Since the initial authorizations of these vaccines, we have learned that protection wanes over time, especially as the virus rapidly mutates and new variants and subvariants emerge,” he said. “Therefore, it’s important to continue discussions about the optimal composition of COVID-19 vaccines for primary and booster vaccination, as well as the optimal interval for booster vaccination.”

A version of this article first appeared on WebMD.com.

The percentage of people in the U.S. getting the latest COVID-19 booster shot has crept up by single digits in the past couple months, despite health officials pleading for people to do so before the Christmas holiday. 

Now, a new poll shows why so few people are willing to roll up their sleeves again.

The most common reasons people give for not getting the latest booster shot is that they “don’t think they need one” (44%) and they “don’t think the benefits are worth it” (37%), according to poll results from the Kaiser Family Foundation. 

The data comes amid announcements by the Centers for Disease Control and Prevention that boosters reduced COVID-19 hospitalizations by up to 57% for U.S. adults and by up to 84% for people age 65 and older. Those figures are just the latest in a mountain of research reporting the public health benefits of COVID-19 vaccines.

Despite all of the statistical data, health officials’ recent vaccination campaigns have proven far from compelling. 

So far, just 15% of people age 12 and older have gotten the latest booster, and 36% of people age 65 and older have gotten it, the CDC’s vaccination trackershows.

Since the start of the pandemic, 1.1 million people in the U.S. have died from COVID-19, with the number of deaths currently rising by 400 per day, The New York Times COVID tracker shows.

Many experts continue to note the need for everyone to get booster shots regularly, but some advocate that perhaps a change in strategy is in order.

“What the administration should do is push for vaccinating people in high-risk groups, including those who are older, those who are immunocompromised and those who have comorbidities,” Paul Offitt, MD, director of the Vaccine Education Center at Children’s Hospital of Philadelphia, told CNN.

Federal regulators have announced they will meet Jan. 26 with a panel of vaccine advisors to examine the current recommended vaccination schedule as well as look at the effectiveness and composition of current vaccines and boosters, with an eye toward the make-up of next-generation shots.

Vaccines are the “best available protection” against hospitalization and death caused by COVID-19, said Peter Marks, MD, PhD, director of the FDA’s Center for Biologics Evaluation and Research, in a statement announcing the planned meeting.

“Since the initial authorizations of these vaccines, we have learned that protection wanes over time, especially as the virus rapidly mutates and new variants and subvariants emerge,” he said. “Therefore, it’s important to continue discussions about the optimal composition of COVID-19 vaccines for primary and booster vaccination, as well as the optimal interval for booster vaccination.”

A version of this article first appeared on WebMD.com.

The percentage of people in the U.S. getting the latest COVID-19 booster shot has crept up by single digits in the past couple months, despite health officials pleading for people to do so before the Christmas holiday. 

Now, a new poll shows why so few people are willing to roll up their sleeves again.

The most common reasons people give for not getting the latest booster shot is that they “don’t think they need one” (44%) and they “don’t think the benefits are worth it” (37%), according to poll results from the Kaiser Family Foundation. 

The data comes amid announcements by the Centers for Disease Control and Prevention that boosters reduced COVID-19 hospitalizations by up to 57% for U.S. adults and by up to 84% for people age 65 and older. Those figures are just the latest in a mountain of research reporting the public health benefits of COVID-19 vaccines.

Despite all of the statistical data, health officials’ recent vaccination campaigns have proven far from compelling. 

So far, just 15% of people age 12 and older have gotten the latest booster, and 36% of people age 65 and older have gotten it, the CDC’s vaccination trackershows.

Since the start of the pandemic, 1.1 million people in the U.S. have died from COVID-19, with the number of deaths currently rising by 400 per day, The New York Times COVID tracker shows.

Many experts continue to note the need for everyone to get booster shots regularly, but some advocate that perhaps a change in strategy is in order.

“What the administration should do is push for vaccinating people in high-risk groups, including those who are older, those who are immunocompromised and those who have comorbidities,” Paul Offitt, MD, director of the Vaccine Education Center at Children’s Hospital of Philadelphia, told CNN.

Federal regulators have announced they will meet Jan. 26 with a panel of vaccine advisors to examine the current recommended vaccination schedule as well as look at the effectiveness and composition of current vaccines and boosters, with an eye toward the make-up of next-generation shots.

Vaccines are the “best available protection” against hospitalization and death caused by COVID-19, said Peter Marks, MD, PhD, director of the FDA’s Center for Biologics Evaluation and Research, in a statement announcing the planned meeting.

“Since the initial authorizations of these vaccines, we have learned that protection wanes over time, especially as the virus rapidly mutates and new variants and subvariants emerge,” he said. “Therefore, it’s important to continue discussions about the optimal composition of COVID-19 vaccines for primary and booster vaccination, as well as the optimal interval for booster vaccination.”

A version of this article first appeared on WebMD.com.

Key clinical point: Patients with moderate-to-severe atopic dermatitis (AD) had the highest incidence rate (IR) per 1000 person-years for major adverse cardiovascular events (MACE), followed by venous thrombotic events (VTE), deep vein thrombosis (DVT), and pulmonary embolism (PE).

Major finding: The IR per 1000 person-years for MACE, VTE, DVT, and PE were 2.6 (95% CI 2.1-3.2), 2.0 (95% CI 1.5-2.5), 1.6 (95% CI 1.2-2.1), and 0.7 (95% CI 0.5-1.0), respectively.

Study details: This retrospective cohort study included 8197 patients aged ≥12 years with moderate-to-severe AD.

Disclosures: This study was funded by Pfizer, Inc. Some authors declared receiving grant funding from Pfizer. Two authors declared being current or former employees and shareholders of Pfizer.

Source: Hedderson MM et al. Rates of cardiovascular events among patients with moderate-to-severe atopic dermatitis in an integrated health care system: A retrospective cohort study. PLoS One. 2022;17(11):e0277469 (Nov 17). Doi: 10.1371/journal.pone.0277469

Key clinical point: Patients with moderate-to-severe atopic dermatitis (AD) had the highest incidence rate (IR) per 1000 person-years for major adverse cardiovascular events (MACE), followed by venous thrombotic events (VTE), deep vein thrombosis (DVT), and pulmonary embolism (PE).

Major finding: The IR per 1000 person-years for MACE, VTE, DVT, and PE were 2.6 (95% CI 2.1-3.2), 2.0 (95% CI 1.5-2.5), 1.6 (95% CI 1.2-2.1), and 0.7 (95% CI 0.5-1.0), respectively.

Study details: This retrospective cohort study included 8197 patients aged ≥12 years with moderate-to-severe AD.

Disclosures: This study was funded by Pfizer, Inc. Some authors declared receiving grant funding from Pfizer. Two authors declared being current or former employees and shareholders of Pfizer.

Source: Hedderson MM et al. Rates of cardiovascular events among patients with moderate-to-severe atopic dermatitis in an integrated health care system: A retrospective cohort study. PLoS One. 2022;17(11):e0277469 (Nov 17). Doi: 10.1371/journal.pone.0277469

Key clinical point: Patients with moderate-to-severe atopic dermatitis (AD) had the highest incidence rate (IR) per 1000 person-years for major adverse cardiovascular events (MACE), followed by venous thrombotic events (VTE), deep vein thrombosis (DVT), and pulmonary embolism (PE).

Major finding: The IR per 1000 person-years for MACE, VTE, DVT, and PE were 2.6 (95% CI 2.1-3.2), 2.0 (95% CI 1.5-2.5), 1.6 (95% CI 1.2-2.1), and 0.7 (95% CI 0.5-1.0), respectively.

Study details: This retrospective cohort study included 8197 patients aged ≥12 years with moderate-to-severe AD.

Disclosures: This study was funded by Pfizer, Inc. Some authors declared receiving grant funding from Pfizer. Two authors declared being current or former employees and shareholders of Pfizer.

Source: Hedderson MM et al. Rates of cardiovascular events among patients with moderate-to-severe atopic dermatitis in an integrated health care system: A retrospective cohort study. PLoS One. 2022;17(11):e0277469 (Nov 17). Doi: 10.1371/journal.pone.0277469

Key clinical point: Baricitinib could serve as an effective treatment option for patients with difficult-to-treat moderate-to-severe atopic dermatitis (AD), including those unresponsive to dupilumab treatment; however, a high discontinuation rate indicates its rather heterogenous efficacy.

Major finding: At week 16, the mean Eczema Area and Severity Index score and numerical rating scale-pruritis significantly decreased from 18.3 to 11.1 (P < .0001) and from 6.6 to 5.3 (P < .0001), respectively. The most frequent adverse events (AE) were nausea (11.8%), urinary tract infection (9.8%), and herpes simplex infections (7.8%). Baricitinib treatment was discontinued by 43.2% of patients due to ineffectiveness or AE.

Study details: This multicenter prospective observational study included 51 adult patients with moderate-to-severe AD from the BioDay registry who received baricitinib over 16 weeks.

Disclosures: This study did not report a source of funding. Some authors declared serving as speakers, consultants, advisory board members, or investigators for various organizations.

Source: Boesjes CM et al. Daily practice experience of baricitinib treatment for patients with difficult-to-treat atopic dermatitis: Results from the BioDay registry. Acta Derm Venereol. 2022;102:adv00820 (Nov 24). Doi: 10.2340/actadv.v102.3978

Key clinical point: Baricitinib could serve as an effective treatment option for patients with difficult-to-treat moderate-to-severe atopic dermatitis (AD), including those unresponsive to dupilumab treatment; however, a high discontinuation rate indicates its rather heterogenous efficacy.

Major finding: At week 16, the mean Eczema Area and Severity Index score and numerical rating scale-pruritis significantly decreased from 18.3 to 11.1 (P < .0001) and from 6.6 to 5.3 (P < .0001), respectively. The most frequent adverse events (AE) were nausea (11.8%), urinary tract infection (9.8%), and herpes simplex infections (7.8%). Baricitinib treatment was discontinued by 43.2% of patients due to ineffectiveness or AE.

Study details: This multicenter prospective observational study included 51 adult patients with moderate-to-severe AD from the BioDay registry who received baricitinib over 16 weeks.

Disclosures: This study did not report a source of funding. Some authors declared serving as speakers, consultants, advisory board members, or investigators for various organizations.

Source: Boesjes CM et al. Daily practice experience of baricitinib treatment for patients with difficult-to-treat atopic dermatitis: Results from the BioDay registry. Acta Derm Venereol. 2022;102:adv00820 (Nov 24). Doi: 10.2340/actadv.v102.3978

Key clinical point: Baricitinib could serve as an effective treatment option for patients with difficult-to-treat moderate-to-severe atopic dermatitis (AD), including those unresponsive to dupilumab treatment; however, a high discontinuation rate indicates its rather heterogenous efficacy.

Major finding: At week 16, the mean Eczema Area and Severity Index score and numerical rating scale-pruritis significantly decreased from 18.3 to 11.1 (P < .0001) and from 6.6 to 5.3 (P < .0001), respectively. The most frequent adverse events (AE) were nausea (11.8%), urinary tract infection (9.8%), and herpes simplex infections (7.8%). Baricitinib treatment was discontinued by 43.2% of patients due to ineffectiveness or AE.

Study details: This multicenter prospective observational study included 51 adult patients with moderate-to-severe AD from the BioDay registry who received baricitinib over 16 weeks.

Disclosures: This study did not report a source of funding. Some authors declared serving as speakers, consultants, advisory board members, or investigators for various organizations.

Source: Boesjes CM et al. Daily practice experience of baricitinib treatment for patients with difficult-to-treat atopic dermatitis: Results from the BioDay registry. Acta Derm Venereol. 2022;102:adv00820 (Nov 24). Doi: 10.2340/actadv.v102.3978

Key clinical point: The first exposure of pediatric individuals to general anesthesia (GA) is not associated with an increased or decreased risk of developing atopic dermatitis (AD).

Major finding: Multivariate analysis revealed that individuals who were exposed vs not exposed to GA did not have an increased or decreased risk of developing AD (adjusted hazard ratio 1.03; P  =  .701).

Study details: This retrospective cohort study included pediatric individuals aged ≤18 years who were (n = 7,681) or were not (n = 38,405; control individuals) exposed to GA.

Disclosures: This study was funded by a 2020 Amorepacific (South Korea) grant. The authors declared no  conflicts of interest.

Source: Kim DC et al. No association between first exposure to general anaesthesia and atopic dermatitis in the paediatric population. Acta Derm Venereol. 2022;102:adv00813 (Nov 14). Doi: 10.2340/actadv.v102.2738

Key clinical point: The first exposure of pediatric individuals to general anesthesia (GA) is not associated with an increased or decreased risk of developing atopic dermatitis (AD).

Major finding: Multivariate analysis revealed that individuals who were exposed vs not exposed to GA did not have an increased or decreased risk of developing AD (adjusted hazard ratio 1.03; P  =  .701).

Study details: This retrospective cohort study included pediatric individuals aged ≤18 years who were (n = 7,681) or were not (n = 38,405; control individuals) exposed to GA.

Disclosures: This study was funded by a 2020 Amorepacific (South Korea) grant. The authors declared no  conflicts of interest.

Source: Kim DC et al. No association between first exposure to general anaesthesia and atopic dermatitis in the paediatric population. Acta Derm Venereol. 2022;102:adv00813 (Nov 14). Doi: 10.2340/actadv.v102.2738

Key clinical point: The first exposure of pediatric individuals to general anesthesia (GA) is not associated with an increased or decreased risk of developing atopic dermatitis (AD).

Major finding: Multivariate analysis revealed that individuals who were exposed vs not exposed to GA did not have an increased or decreased risk of developing AD (adjusted hazard ratio 1.03; P  =  .701).

Study details: This retrospective cohort study included pediatric individuals aged ≤18 years who were (n = 7,681) or were not (n = 38,405; control individuals) exposed to GA.

Disclosures: This study was funded by a 2020 Amorepacific (South Korea) grant. The authors declared no  conflicts of interest.

Source: Kim DC et al. No association between first exposure to general anaesthesia and atopic dermatitis in the paediatric population. Acta Derm Venereol. 2022;102:adv00813 (Nov 14). Doi: 10.2340/actadv.v102.2738

Key clinical point: Downtitrated baricitinib treatment was efficacious in most patients with moderate-to-severe atopic dermatitis (AD) through 16 weeks; clinically relevant efficacy was observed in most patients who were readministered the original dose due to downtitration or treatment withdrawal-related efficacy loss.

Major finding: In the 4-mg and 2-mg cohorts, 61% and 71% of patients maintained a validated Investigator’s Global Assessment for AD (vIGA-AD) score of 0/1/2 at downtitration week 16 and 80%/85%/88% and 90%/56%/86% of patients who switched to original dose in the continuous dosing/downtitration/treatment withdrawal group re-achieved vIGA-AD 0/1/2, respectively.

Study details: This BREEZE-AD3 trial substudy included 526 patients with moderate-to-severe AD treated with 4/2 mg baricitinib at trial entry who achieved vIGA-AD 0/1/2 at week 52, with each cohort being re-assigned to continuous dosing, downtitration, or treatment withdrawal.

Disclosures: This study was sponsored by Eli Lilly and Company under license from Incyte Corporation. Some authors reported ties with various sources, including Eli Lilly and Incyte. Three authors declared being current or former employees and shareholders of Eli Lilly.

Source: Reich K et al. Efficacy of downtitration or treatment withdrawal compared to continuous dosing after successful treatment with baricitinib in patients with moderate-to-severe atopic dermatitis in a randomised substudy from the long-term extension study, BREEZE-AD3. Br J Dermatol. 2022 (Nov 17). Doi: 10.1093/bjd/ljac057

Key clinical point: Downtitrated baricitinib treatment was efficacious in most patients with moderate-to-severe atopic dermatitis (AD) through 16 weeks; clinically relevant efficacy was observed in most patients who were readministered the original dose due to downtitration or treatment withdrawal-related efficacy loss.

Major finding: In the 4-mg and 2-mg cohorts, 61% and 71% of patients maintained a validated Investigator’s Global Assessment for AD (vIGA-AD) score of 0/1/2 at downtitration week 16 and 80%/85%/88% and 90%/56%/86% of patients who switched to original dose in the continuous dosing/downtitration/treatment withdrawal group re-achieved vIGA-AD 0/1/2, respectively.

Study details: This BREEZE-AD3 trial substudy included 526 patients with moderate-to-severe AD treated with 4/2 mg baricitinib at trial entry who achieved vIGA-AD 0/1/2 at week 52, with each cohort being re-assigned to continuous dosing, downtitration, or treatment withdrawal.

Disclosures: This study was sponsored by Eli Lilly and Company under license from Incyte Corporation. Some authors reported ties with various sources, including Eli Lilly and Incyte. Three authors declared being current or former employees and shareholders of Eli Lilly.

Source: Reich K et al. Efficacy of downtitration or treatment withdrawal compared to continuous dosing after successful treatment with baricitinib in patients with moderate-to-severe atopic dermatitis in a randomised substudy from the long-term extension study, BREEZE-AD3. Br J Dermatol. 2022 (Nov 17). Doi: 10.1093/bjd/ljac057

Key clinical point: Downtitrated baricitinib treatment was efficacious in most patients with moderate-to-severe atopic dermatitis (AD) through 16 weeks; clinically relevant efficacy was observed in most patients who were readministered the original dose due to downtitration or treatment withdrawal-related efficacy loss.

Major finding: In the 4-mg and 2-mg cohorts, 61% and 71% of patients maintained a validated Investigator’s Global Assessment for AD (vIGA-AD) score of 0/1/2 at downtitration week 16 and 80%/85%/88% and 90%/56%/86% of patients who switched to original dose in the continuous dosing/downtitration/treatment withdrawal group re-achieved vIGA-AD 0/1/2, respectively.

Study details: This BREEZE-AD3 trial substudy included 526 patients with moderate-to-severe AD treated with 4/2 mg baricitinib at trial entry who achieved vIGA-AD 0/1/2 at week 52, with each cohort being re-assigned to continuous dosing, downtitration, or treatment withdrawal.

Disclosures: This study was sponsored by Eli Lilly and Company under license from Incyte Corporation. Some authors reported ties with various sources, including Eli Lilly and Incyte. Three authors declared being current or former employees and shareholders of Eli Lilly.

Source: Reich K et al. Efficacy of downtitration or treatment withdrawal compared to continuous dosing after successful treatment with baricitinib in patients with moderate-to-severe atopic dermatitis in a randomised substudy from the long-term extension study, BREEZE-AD3. Br J Dermatol. 2022 (Nov 17). Doi: 10.1093/bjd/ljac057

Key clinical point: Dupilumab decreases disease severity, disease-associated symptoms, and severity-associated serum biomarker levels in pediatric patients with atopic dermatitis (AD) in daily practice.

Major finding: At week 28, 49.2% of patients achieved Eczema Area and Severity Index-75; 84.7%, 45.3%, and 77.4% achieved a ≥4-point improvement in the Patient-Oriented Eczema Measure, Numeric Rating Scale (NRS)-pruritus, and NRS-pain scores, respectively; 36.1% scored clear or almost clear on the Investigator’s Global Assessment. The levels of severity-associated markers soluble IL-2-receptor alpha (P < .05), periostin (P < .05), thymus- and activation-regulated chemokine (P < .005), and pulmonary- and activation-regulated chemokine (P < .005) were significantly reduced at week 4. Conjunctivitis (16.4%) and headache (6.6%) were the most common side effects.

Study details: This multicenter prospective observational study included 61 patients (children ≥6 to <12 years; adolescents ≥12 to <18 years) from the BioDay registry with moderate-to-severe AD who received dupilumab for 28 weeks.

Disclosures: The BioDay registry is sponsored by Sanofi/Regeneron and others. Some authors reported ties with various sources, including Sanofi.

Source: Kamphuis E, Boesjes CM et al. Dupilumab in daily practice for the treatment of pediatric atopic dermatitis: 28-week clinical and biomarker results from the BioDay registry. Pediatr Allergy Immunol. 2022;13(12):e13887 (Dec 5). Doi: 10.1111/pai.13887

Key clinical point: Dupilumab decreases disease severity, disease-associated symptoms, and severity-associated serum biomarker levels in pediatric patients with atopic dermatitis (AD) in daily practice.

Major finding: At week 28, 49.2% of patients achieved Eczema Area and Severity Index-75; 84.7%, 45.3%, and 77.4% achieved a ≥4-point improvement in the Patient-Oriented Eczema Measure, Numeric Rating Scale (NRS)-pruritus, and NRS-pain scores, respectively; 36.1% scored clear or almost clear on the Investigator’s Global Assessment. The levels of severity-associated markers soluble IL-2-receptor alpha (P < .05), periostin (P < .05), thymus- and activation-regulated chemokine (P < .005), and pulmonary- and activation-regulated chemokine (P < .005) were significantly reduced at week 4. Conjunctivitis (16.4%) and headache (6.6%) were the most common side effects.

Study details: This multicenter prospective observational study included 61 patients (children ≥6 to <12 years; adolescents ≥12 to <18 years) from the BioDay registry with moderate-to-severe AD who received dupilumab for 28 weeks.

Disclosures: The BioDay registry is sponsored by Sanofi/Regeneron and others. Some authors reported ties with various sources, including Sanofi.

Source: Kamphuis E, Boesjes CM et al. Dupilumab in daily practice for the treatment of pediatric atopic dermatitis: 28-week clinical and biomarker results from the BioDay registry. Pediatr Allergy Immunol. 2022;13(12):e13887 (Dec 5). Doi: 10.1111/pai.13887

Key clinical point: Dupilumab decreases disease severity, disease-associated symptoms, and severity-associated serum biomarker levels in pediatric patients with atopic dermatitis (AD) in daily practice.

Major finding: At week 28, 49.2% of patients achieved Eczema Area and Severity Index-75; 84.7%, 45.3%, and 77.4% achieved a ≥4-point improvement in the Patient-Oriented Eczema Measure, Numeric Rating Scale (NRS)-pruritus, and NRS-pain scores, respectively; 36.1% scored clear or almost clear on the Investigator’s Global Assessment. The levels of severity-associated markers soluble IL-2-receptor alpha (P < .05), periostin (P < .05), thymus- and activation-regulated chemokine (P < .005), and pulmonary- and activation-regulated chemokine (P < .005) were significantly reduced at week 4. Conjunctivitis (16.4%) and headache (6.6%) were the most common side effects.

Study details: This multicenter prospective observational study included 61 patients (children ≥6 to <12 years; adolescents ≥12 to <18 years) from the BioDay registry with moderate-to-severe AD who received dupilumab for 28 weeks.

Disclosures: The BioDay registry is sponsored by Sanofi/Regeneron and others. Some authors reported ties with various sources, including Sanofi.

Source: Kamphuis E, Boesjes CM et al. Dupilumab in daily practice for the treatment of pediatric atopic dermatitis: 28-week clinical and biomarker results from the BioDay registry. Pediatr Allergy Immunol. 2022;13(12):e13887 (Dec 5). Doi: 10.1111/pai.13887

Key clinical point: Early application of emollients can effectively prevent atopic dermatitis (AD) in infants, with emollient emulsion seeming an optimal treatment option in infancy compared with creams or mixed emollients.

Major finding: The incidence of AD was significantly lower in high-risk infants receiving early emollients vs standard care (risk ratio 0.64; 95% CI 0.47-0.88), with surface under the cumulative ranking curve analysis revealing emollient emulsion (82.6%) as the optimal treatment for AD prevention in infants, followed by mixed emollient (77.4%) and emollient cream (21.9%).

Study details: This was a systematic review and network meta-analysis of 11 randomized controlled trials including 3483 infants without AD who received either prophylactic emollients (cream, emulsion, or mixed types) or standard care.

Disclosures: This study was supported by the Key Research and Development Project of Xinjiang Uygur Autonomous Region, China. The authors declared no  conflicts of interest.

Source: Liang J, Hu F et al. Systematic review and network meta‐analysis of different types of emollient for the prevention of atopic dermatitis in infants. J Eur Acad Dermatol Venereol. 2022 (Nov 23). Doi: 10.1111/jdv.18688

Key clinical point: Early application of emollients can effectively prevent atopic dermatitis (AD) in infants, with emollient emulsion seeming an optimal treatment option in infancy compared with creams or mixed emollients.

Major finding: The incidence of AD was significantly lower in high-risk infants receiving early emollients vs standard care (risk ratio 0.64; 95% CI 0.47-0.88), with surface under the cumulative ranking curve analysis revealing emollient emulsion (82.6%) as the optimal treatment for AD prevention in infants, followed by mixed emollient (77.4%) and emollient cream (21.9%).

Study details: This was a systematic review and network meta-analysis of 11 randomized controlled trials including 3483 infants without AD who received either prophylactic emollients (cream, emulsion, or mixed types) or standard care.

Disclosures: This study was supported by the Key Research and Development Project of Xinjiang Uygur Autonomous Region, China. The authors declared no  conflicts of interest.

Source: Liang J, Hu F et al. Systematic review and network meta‐analysis of different types of emollient for the prevention of atopic dermatitis in infants. J Eur Acad Dermatol Venereol. 2022 (Nov 23). Doi: 10.1111/jdv.18688

Key clinical point: Early application of emollients can effectively prevent atopic dermatitis (AD) in infants, with emollient emulsion seeming an optimal treatment option in infancy compared with creams or mixed emollients.

Major finding: The incidence of AD was significantly lower in high-risk infants receiving early emollients vs standard care (risk ratio 0.64; 95% CI 0.47-0.88), with surface under the cumulative ranking curve analysis revealing emollient emulsion (82.6%) as the optimal treatment for AD prevention in infants, followed by mixed emollient (77.4%) and emollient cream (21.9%).

Study details: This was a systematic review and network meta-analysis of 11 randomized controlled trials including 3483 infants without AD who received either prophylactic emollients (cream, emulsion, or mixed types) or standard care.

Disclosures: This study was supported by the Key Research and Development Project of Xinjiang Uygur Autonomous Region, China. The authors declared no  conflicts of interest.

Source: Liang J, Hu F et al. Systematic review and network meta‐analysis of different types of emollient for the prevention of atopic dermatitis in infants. J Eur Acad Dermatol Venereol. 2022 (Nov 23). Doi: 10.1111/jdv.18688

Key clinical point: Ruxolitinib cream demonstrated effective disease control and was well tolerated in patients with atopic dermatitis (AD) during 44 weeks of as-needed treatment.

Major finding: At week 52, 74.1%-77.8% of patients had an Investigator’s Global Assessment score of 0/1, with the mean affected body surface area being 1.4%-1.8%. Treatment-related adverse events were reported in 8.7%/7.4% of patients on 0.75%/1.5% ruxolitinib and in 2.0%/6.1% of those who switched from vehicle to 0.75%/1.5% ruxolitinib, respectively.

Study details: This study analyzed pooled data from two phase 3 studies, TRuE-AD1 and TRuE-AD2, including 1249 patients aged ≥12 years with AD who were randomly assigned to receive 0.75% or 1.5% ruxolitinib cream or vehicle for 8 weeks; thereafter, the vehicle group patients were re-assigned to receive either strength ruxolitinib cream for 44 weeks.

Disclosures: This study was funded by Incyte Corporation, U.S. Some authors reported ties with various sources, including Incyte. Four authors declared being current or former employees and shareholders of Incyte.

Source: Papp K et al. Long-term safety and disease control with ruxolitinib cream in atopic dermatitis: Results from two phase 3 studies. J Am Acad Dermatol. 2022 (Nov 25). Doi: 10.1016/j.jaad.2022.09.060

Key clinical point: Ruxolitinib cream demonstrated effective disease control and was well tolerated in patients with atopic dermatitis (AD) during 44 weeks of as-needed treatment.

Major finding: At week 52, 74.1%-77.8% of patients had an Investigator’s Global Assessment score of 0/1, with the mean affected body surface area being 1.4%-1.8%. Treatment-related adverse events were reported in 8.7%/7.4% of patients on 0.75%/1.5% ruxolitinib and in 2.0%/6.1% of those who switched from vehicle to 0.75%/1.5% ruxolitinib, respectively.

Study details: This study analyzed pooled data from two phase 3 studies, TRuE-AD1 and TRuE-AD2, including 1249 patients aged ≥12 years with AD who were randomly assigned to receive 0.75% or 1.5% ruxolitinib cream or vehicle for 8 weeks; thereafter, the vehicle group patients were re-assigned to receive either strength ruxolitinib cream for 44 weeks.

Disclosures: This study was funded by Incyte Corporation, U.S. Some authors reported ties with various sources, including Incyte. Four authors declared being current or former employees and shareholders of Incyte.

Source: Papp K et al. Long-term safety and disease control with ruxolitinib cream in atopic dermatitis: Results from two phase 3 studies. J Am Acad Dermatol. 2022 (Nov 25). Doi: 10.1016/j.jaad.2022.09.060

Key clinical point: Ruxolitinib cream demonstrated effective disease control and was well tolerated in patients with atopic dermatitis (AD) during 44 weeks of as-needed treatment.

Major finding: At week 52, 74.1%-77.8% of patients had an Investigator’s Global Assessment score of 0/1, with the mean affected body surface area being 1.4%-1.8%. Treatment-related adverse events were reported in 8.7%/7.4% of patients on 0.75%/1.5% ruxolitinib and in 2.0%/6.1% of those who switched from vehicle to 0.75%/1.5% ruxolitinib, respectively.

Study details: This study analyzed pooled data from two phase 3 studies, TRuE-AD1 and TRuE-AD2, including 1249 patients aged ≥12 years with AD who were randomly assigned to receive 0.75% or 1.5% ruxolitinib cream or vehicle for 8 weeks; thereafter, the vehicle group patients were re-assigned to receive either strength ruxolitinib cream for 44 weeks.

Disclosures: This study was funded by Incyte Corporation, U.S. Some authors reported ties with various sources, including Incyte. Four authors declared being current or former employees and shareholders of Incyte.

Source: Papp K et al. Long-term safety and disease control with ruxolitinib cream in atopic dermatitis: Results from two phase 3 studies. J Am Acad Dermatol. 2022 (Nov 25). Doi: 10.1016/j.jaad.2022.09.060