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30 years of fake nursing ends with 7-year prison sentence
A Canadian woman who officials allege faked being a registered nurse for some 30 years in Canada and the United States is scheduled to appear in court next month after being sentenced to 7 years in prison.
pled guilty in January to seven offenses, including impersonation, assault with a weapon, and assault, according to CBC Radio-Canada.
Ms. Cleroux, who uses several aliases, had a long history of deception in three provinces in Canada, as well as in Colorado and Florida. The sentencing in Ontario stemmed from incidents at a medical and dental clinic in Ottawa last year, which included administration of medications to patients through needle injections, Ottawa Police reported in a press statement obtained by this news organization.
Authorities charged Ms. Cleroux in September with assault with a weapon and criminal negligence causing bodily harm, along with “personation to gain advantage,” obtaining by false pretense, and using a forged document, this news organization reported.
Ms. Cleroux has been in custody since her arrest by Ottawa Police in August.
The Vancouver Police Department (VPD) charged Ms. Cleroux last year with fraud of over $5,000 and personation with intent. VPD investigated claims that an employee at BC Women’s Hospital fraudulently identified herself as a nurse while working there between June 2020 and June 2021, according to a VPD press release.
Nursing colleges in British Columbia and Ontario issued warnings that she had used aliases and purported to be a registered nurse to gain employment. The aliases included Melanie Thompson, Melanie Smith, and Melanie Cleroux.
Ms. Cleroux was believed to be a student in a nursing school in Colorado, but she only completed 2 years of a 4-year nursing course and was never certified as a nurse, according to CBC. Her criminal record dates back 30 years and includes 67 adult convictions and other convictions in her youth, CBC reported.
A version of this article first appeared on Medscape.com.
A Canadian woman who officials allege faked being a registered nurse for some 30 years in Canada and the United States is scheduled to appear in court next month after being sentenced to 7 years in prison.
pled guilty in January to seven offenses, including impersonation, assault with a weapon, and assault, according to CBC Radio-Canada.
Ms. Cleroux, who uses several aliases, had a long history of deception in three provinces in Canada, as well as in Colorado and Florida. The sentencing in Ontario stemmed from incidents at a medical and dental clinic in Ottawa last year, which included administration of medications to patients through needle injections, Ottawa Police reported in a press statement obtained by this news organization.
Authorities charged Ms. Cleroux in September with assault with a weapon and criminal negligence causing bodily harm, along with “personation to gain advantage,” obtaining by false pretense, and using a forged document, this news organization reported.
Ms. Cleroux has been in custody since her arrest by Ottawa Police in August.
The Vancouver Police Department (VPD) charged Ms. Cleroux last year with fraud of over $5,000 and personation with intent. VPD investigated claims that an employee at BC Women’s Hospital fraudulently identified herself as a nurse while working there between June 2020 and June 2021, according to a VPD press release.
Nursing colleges in British Columbia and Ontario issued warnings that she had used aliases and purported to be a registered nurse to gain employment. The aliases included Melanie Thompson, Melanie Smith, and Melanie Cleroux.
Ms. Cleroux was believed to be a student in a nursing school in Colorado, but she only completed 2 years of a 4-year nursing course and was never certified as a nurse, according to CBC. Her criminal record dates back 30 years and includes 67 adult convictions and other convictions in her youth, CBC reported.
A version of this article first appeared on Medscape.com.
A Canadian woman who officials allege faked being a registered nurse for some 30 years in Canada and the United States is scheduled to appear in court next month after being sentenced to 7 years in prison.
pled guilty in January to seven offenses, including impersonation, assault with a weapon, and assault, according to CBC Radio-Canada.
Ms. Cleroux, who uses several aliases, had a long history of deception in three provinces in Canada, as well as in Colorado and Florida. The sentencing in Ontario stemmed from incidents at a medical and dental clinic in Ottawa last year, which included administration of medications to patients through needle injections, Ottawa Police reported in a press statement obtained by this news organization.
Authorities charged Ms. Cleroux in September with assault with a weapon and criminal negligence causing bodily harm, along with “personation to gain advantage,” obtaining by false pretense, and using a forged document, this news organization reported.
Ms. Cleroux has been in custody since her arrest by Ottawa Police in August.
The Vancouver Police Department (VPD) charged Ms. Cleroux last year with fraud of over $5,000 and personation with intent. VPD investigated claims that an employee at BC Women’s Hospital fraudulently identified herself as a nurse while working there between June 2020 and June 2021, according to a VPD press release.
Nursing colleges in British Columbia and Ontario issued warnings that she had used aliases and purported to be a registered nurse to gain employment. The aliases included Melanie Thompson, Melanie Smith, and Melanie Cleroux.
Ms. Cleroux was believed to be a student in a nursing school in Colorado, but she only completed 2 years of a 4-year nursing course and was never certified as a nurse, according to CBC. Her criminal record dates back 30 years and includes 67 adult convictions and other convictions in her youth, CBC reported.
A version of this article first appeared on Medscape.com.
Nap length linked to cognitive changes
No wonder we feel worse after naps
Some of us have hectic schedules that may make a nap feel more necessary. It’s common knowledge that naps shouldn’t be too long – maybe 20 minutes or so – but if you frequently take 3-hour naps and wake up thinking you’re late for school even though you’re 47 and have your PhD, this LOTME is for you.
Studies have shown that there is a link between napping during the day and Alzheimer’s/cognitive decline, but now we’ve got a double whammy for you: Longer and more frequent napping is linked to worse cognition after a year, and in turn, those with cognitive decline and Alzheimer’s are known to nap longer and more frequently during the day.
“We now know that the pathology related to cognitive decline can cause other changes in function,” he said. “It’s really a multisystem disorder, also including difficulty sleeping, changes in movement, changes in body composition, depression symptoms, behavioral changes, etc.,” coauthor Aron Buchman, MD, said in a statement from Rush University Medical Center.
The investigators monitored 1,400 patients over the course of 14 years with wrist bracelets that recorded when a person was not active during the day and considered that a nap.
At the beginning of the study, 75% of the study subjects had no cognitive impairment, 19.5% had some cognitive impairment, and approximately 4% had Alzheimer’s. Napping during the day only increased about 11 minutes a year for those with no signs of cognitive impairment, but those who showed significantly more signs of cognitive decline doubled their nap time and those actually diagnosed with Alzheimer’s tripled theirs.
The investigators did not imply that napping causes Alzheimer’s, but they noted that people who are older and nap more than an hour a day are 40% more likely to be at risk. It is something to consider and monitor.
Sometimes, after all, a nap seems like the best idea ever, but more often than not we wake up feeling 10 times worse. Our bodies may be giving us a heads up.
Pokemon Go away depression
The summer of 2016 was a great time if you happened to be a fan of Pokemon. Which is quite a lot of people. For almost 20 years millions have enjoyed the games and animated series, but Pokemon Go brought the thrill of catching Pokemon to life in a whole new way. For the first time, you could go out into the world and pretend you were a real Pokemon trainer, and everywhere you went, there would be others like you.
The ability to chase after Pikachu and Charizard in real life (well, augmented reality, but close enough) seemed to bring people a lot of joy, but seemed is never good enough for science. Can’t have anecdotes, we need data! So researchers at the London School of Economics and Political Science conducted a study into how Pokemon Go affected local Internet search rates of depression as the game was released slowly around the world.
Through analyzing Google Trend data of words like “depression,” “anxiety,” and “stress,” the researchers found that the release of Pokemon Go was significantly associated with a noticeable, though short-term, drop in depression-related Internet searches. Location-based augmented reality games may alleviate symptoms of mild depression, the researchers said, as they encourage physical activity, face-to-face socialization, and exposure to nature, though they added that simply going outside is likely not enough to combat clinical cases of severe depression.
Still, augmented reality games represent a viable target for public health investment, since they’re easy to use and inexpensive to make. That said, we’re not sure we want the FDA or CDC making a new Pokemon Go game. They’d probably end up filling the streets with Mr. Mime. And no one would leave their house for that.
And now a word from our sponsor
How many times has this happened to you? You need to repair a jet engine, inspect a nuclear reactor cooling system, AND perform bowel surgery, but you can’t carry around all the heavy, old-fashioned tools needed for those jobs.
Well, we’ve got one tool that can do it all! And that tool is a snake. No, it’s a robot.
It’s both! It’s the COntinuum roBot for Remote Applications. COBRA is the robot that looks like a snake! A snake that’s 5 meters long but only as thick as a pencil (about 9 mm in diameter). A robot with “extraordinary manoeuvrability and responsiveness due to … a compliant-joint structure and multiple continuous sections that enable it to bend at around 90 degrees,” according to the team at the University of Nottingham (England) that developed it.
COBRA comes equipped with a stereovision camera and a miniature cutting tool to perform complex industrial repair, but other devices can be interchanged for possible medical use.
COBRA and its joystick-like controller were designed to be easy to use. Dr. Oladejo Olaleye, the ear, nose, and throat and robotic surgeon at University Hospitals of Leicester who is directing its surgical development, was able to use COBRA on a dummy after just 5 minutes of training. He called it “the future of diagnostic endoscopy and therapeutic surgery.”
Don’t be the last aircraft engineer/nuclear technician/surgeon on your block to have this ultraslender, ultramaneuverable reptilian repair robot. Get your COBRA now! Operators are standing by.
Disclaimer: Robot is still under development and not yet on sale.
Rule, (worm) Britannia!
As long as there have been people, there have been parasitic worms living in their guts. Helminth infection is a continuing and largely ignored crisis in poor, tropical nations, though worm-based diseases have been basically eliminated from wealthier countries.
This wasn’t always the case, however, as a study published in PLOS Neglected Tropical Diseases (now there’s a specific topic) has found. The researchers detail the glorious history of helminth infestation in the United Kingdom from the Victorian era all the way back to prehistory, scouring hundreds of skeletons found in 17 sites across the country for eggs, which can remain intact for thousands of years.
The researchers found that two eras in particular had very high rates of infection. Unsurprisingly, the late medieval era was one of them, but the other is less obvious. The Romans were famous for their hygiene, their baths, and their plumbing, but maybe they also should be famous for the abundance of worms in their bellies. That doesn’t make sense at first: Shouldn’t good hygiene lower infection? The benefits of a good sewer system, however, are lessened when the waste containing said infectious organisms is used to fertilize crops. Recycling is generally a good thing, but less so when you’re recycling parasitic worms.
Curiously, of the three sites from the industrial age, only the one in London had high levels of worm infestation. Considering how dirty and cramped 19th-century British cities were, one might expect disease to run rampant (tuberculosis certainly did), but the sites in Oxford and Birmingham were almost devoid of worms. The researchers theorized that this was because of access to clean well water. Or maybe worms just have a thing for London. [Editor’s note: It’s probably not that.]
No wonder we feel worse after naps
Some of us have hectic schedules that may make a nap feel more necessary. It’s common knowledge that naps shouldn’t be too long – maybe 20 minutes or so – but if you frequently take 3-hour naps and wake up thinking you’re late for school even though you’re 47 and have your PhD, this LOTME is for you.
Studies have shown that there is a link between napping during the day and Alzheimer’s/cognitive decline, but now we’ve got a double whammy for you: Longer and more frequent napping is linked to worse cognition after a year, and in turn, those with cognitive decline and Alzheimer’s are known to nap longer and more frequently during the day.
“We now know that the pathology related to cognitive decline can cause other changes in function,” he said. “It’s really a multisystem disorder, also including difficulty sleeping, changes in movement, changes in body composition, depression symptoms, behavioral changes, etc.,” coauthor Aron Buchman, MD, said in a statement from Rush University Medical Center.
The investigators monitored 1,400 patients over the course of 14 years with wrist bracelets that recorded when a person was not active during the day and considered that a nap.
At the beginning of the study, 75% of the study subjects had no cognitive impairment, 19.5% had some cognitive impairment, and approximately 4% had Alzheimer’s. Napping during the day only increased about 11 minutes a year for those with no signs of cognitive impairment, but those who showed significantly more signs of cognitive decline doubled their nap time and those actually diagnosed with Alzheimer’s tripled theirs.
The investigators did not imply that napping causes Alzheimer’s, but they noted that people who are older and nap more than an hour a day are 40% more likely to be at risk. It is something to consider and monitor.
Sometimes, after all, a nap seems like the best idea ever, but more often than not we wake up feeling 10 times worse. Our bodies may be giving us a heads up.
Pokemon Go away depression
The summer of 2016 was a great time if you happened to be a fan of Pokemon. Which is quite a lot of people. For almost 20 years millions have enjoyed the games and animated series, but Pokemon Go brought the thrill of catching Pokemon to life in a whole new way. For the first time, you could go out into the world and pretend you were a real Pokemon trainer, and everywhere you went, there would be others like you.
The ability to chase after Pikachu and Charizard in real life (well, augmented reality, but close enough) seemed to bring people a lot of joy, but seemed is never good enough for science. Can’t have anecdotes, we need data! So researchers at the London School of Economics and Political Science conducted a study into how Pokemon Go affected local Internet search rates of depression as the game was released slowly around the world.
Through analyzing Google Trend data of words like “depression,” “anxiety,” and “stress,” the researchers found that the release of Pokemon Go was significantly associated with a noticeable, though short-term, drop in depression-related Internet searches. Location-based augmented reality games may alleviate symptoms of mild depression, the researchers said, as they encourage physical activity, face-to-face socialization, and exposure to nature, though they added that simply going outside is likely not enough to combat clinical cases of severe depression.
Still, augmented reality games represent a viable target for public health investment, since they’re easy to use and inexpensive to make. That said, we’re not sure we want the FDA or CDC making a new Pokemon Go game. They’d probably end up filling the streets with Mr. Mime. And no one would leave their house for that.
And now a word from our sponsor
How many times has this happened to you? You need to repair a jet engine, inspect a nuclear reactor cooling system, AND perform bowel surgery, but you can’t carry around all the heavy, old-fashioned tools needed for those jobs.
Well, we’ve got one tool that can do it all! And that tool is a snake. No, it’s a robot.
It’s both! It’s the COntinuum roBot for Remote Applications. COBRA is the robot that looks like a snake! A snake that’s 5 meters long but only as thick as a pencil (about 9 mm in diameter). A robot with “extraordinary manoeuvrability and responsiveness due to … a compliant-joint structure and multiple continuous sections that enable it to bend at around 90 degrees,” according to the team at the University of Nottingham (England) that developed it.
COBRA comes equipped with a stereovision camera and a miniature cutting tool to perform complex industrial repair, but other devices can be interchanged for possible medical use.
COBRA and its joystick-like controller were designed to be easy to use. Dr. Oladejo Olaleye, the ear, nose, and throat and robotic surgeon at University Hospitals of Leicester who is directing its surgical development, was able to use COBRA on a dummy after just 5 minutes of training. He called it “the future of diagnostic endoscopy and therapeutic surgery.”
Don’t be the last aircraft engineer/nuclear technician/surgeon on your block to have this ultraslender, ultramaneuverable reptilian repair robot. Get your COBRA now! Operators are standing by.
Disclaimer: Robot is still under development and not yet on sale.
Rule, (worm) Britannia!
As long as there have been people, there have been parasitic worms living in their guts. Helminth infection is a continuing and largely ignored crisis in poor, tropical nations, though worm-based diseases have been basically eliminated from wealthier countries.
This wasn’t always the case, however, as a study published in PLOS Neglected Tropical Diseases (now there’s a specific topic) has found. The researchers detail the glorious history of helminth infestation in the United Kingdom from the Victorian era all the way back to prehistory, scouring hundreds of skeletons found in 17 sites across the country for eggs, which can remain intact for thousands of years.
The researchers found that two eras in particular had very high rates of infection. Unsurprisingly, the late medieval era was one of them, but the other is less obvious. The Romans were famous for their hygiene, their baths, and their plumbing, but maybe they also should be famous for the abundance of worms in their bellies. That doesn’t make sense at first: Shouldn’t good hygiene lower infection? The benefits of a good sewer system, however, are lessened when the waste containing said infectious organisms is used to fertilize crops. Recycling is generally a good thing, but less so when you’re recycling parasitic worms.
Curiously, of the three sites from the industrial age, only the one in London had high levels of worm infestation. Considering how dirty and cramped 19th-century British cities were, one might expect disease to run rampant (tuberculosis certainly did), but the sites in Oxford and Birmingham were almost devoid of worms. The researchers theorized that this was because of access to clean well water. Or maybe worms just have a thing for London. [Editor’s note: It’s probably not that.]
No wonder we feel worse after naps
Some of us have hectic schedules that may make a nap feel more necessary. It’s common knowledge that naps shouldn’t be too long – maybe 20 minutes or so – but if you frequently take 3-hour naps and wake up thinking you’re late for school even though you’re 47 and have your PhD, this LOTME is for you.
Studies have shown that there is a link between napping during the day and Alzheimer’s/cognitive decline, but now we’ve got a double whammy for you: Longer and more frequent napping is linked to worse cognition after a year, and in turn, those with cognitive decline and Alzheimer’s are known to nap longer and more frequently during the day.
“We now know that the pathology related to cognitive decline can cause other changes in function,” he said. “It’s really a multisystem disorder, also including difficulty sleeping, changes in movement, changes in body composition, depression symptoms, behavioral changes, etc.,” coauthor Aron Buchman, MD, said in a statement from Rush University Medical Center.
The investigators monitored 1,400 patients over the course of 14 years with wrist bracelets that recorded when a person was not active during the day and considered that a nap.
At the beginning of the study, 75% of the study subjects had no cognitive impairment, 19.5% had some cognitive impairment, and approximately 4% had Alzheimer’s. Napping during the day only increased about 11 minutes a year for those with no signs of cognitive impairment, but those who showed significantly more signs of cognitive decline doubled their nap time and those actually diagnosed with Alzheimer’s tripled theirs.
The investigators did not imply that napping causes Alzheimer’s, but they noted that people who are older and nap more than an hour a day are 40% more likely to be at risk. It is something to consider and monitor.
Sometimes, after all, a nap seems like the best idea ever, but more often than not we wake up feeling 10 times worse. Our bodies may be giving us a heads up.
Pokemon Go away depression
The summer of 2016 was a great time if you happened to be a fan of Pokemon. Which is quite a lot of people. For almost 20 years millions have enjoyed the games and animated series, but Pokemon Go brought the thrill of catching Pokemon to life in a whole new way. For the first time, you could go out into the world and pretend you were a real Pokemon trainer, and everywhere you went, there would be others like you.
The ability to chase after Pikachu and Charizard in real life (well, augmented reality, but close enough) seemed to bring people a lot of joy, but seemed is never good enough for science. Can’t have anecdotes, we need data! So researchers at the London School of Economics and Political Science conducted a study into how Pokemon Go affected local Internet search rates of depression as the game was released slowly around the world.
Through analyzing Google Trend data of words like “depression,” “anxiety,” and “stress,” the researchers found that the release of Pokemon Go was significantly associated with a noticeable, though short-term, drop in depression-related Internet searches. Location-based augmented reality games may alleviate symptoms of mild depression, the researchers said, as they encourage physical activity, face-to-face socialization, and exposure to nature, though they added that simply going outside is likely not enough to combat clinical cases of severe depression.
Still, augmented reality games represent a viable target for public health investment, since they’re easy to use and inexpensive to make. That said, we’re not sure we want the FDA or CDC making a new Pokemon Go game. They’d probably end up filling the streets with Mr. Mime. And no one would leave their house for that.
And now a word from our sponsor
How many times has this happened to you? You need to repair a jet engine, inspect a nuclear reactor cooling system, AND perform bowel surgery, but you can’t carry around all the heavy, old-fashioned tools needed for those jobs.
Well, we’ve got one tool that can do it all! And that tool is a snake. No, it’s a robot.
It’s both! It’s the COntinuum roBot for Remote Applications. COBRA is the robot that looks like a snake! A snake that’s 5 meters long but only as thick as a pencil (about 9 mm in diameter). A robot with “extraordinary manoeuvrability and responsiveness due to … a compliant-joint structure and multiple continuous sections that enable it to bend at around 90 degrees,” according to the team at the University of Nottingham (England) that developed it.
COBRA comes equipped with a stereovision camera and a miniature cutting tool to perform complex industrial repair, but other devices can be interchanged for possible medical use.
COBRA and its joystick-like controller were designed to be easy to use. Dr. Oladejo Olaleye, the ear, nose, and throat and robotic surgeon at University Hospitals of Leicester who is directing its surgical development, was able to use COBRA on a dummy after just 5 minutes of training. He called it “the future of diagnostic endoscopy and therapeutic surgery.”
Don’t be the last aircraft engineer/nuclear technician/surgeon on your block to have this ultraslender, ultramaneuverable reptilian repair robot. Get your COBRA now! Operators are standing by.
Disclaimer: Robot is still under development and not yet on sale.
Rule, (worm) Britannia!
As long as there have been people, there have been parasitic worms living in their guts. Helminth infection is a continuing and largely ignored crisis in poor, tropical nations, though worm-based diseases have been basically eliminated from wealthier countries.
This wasn’t always the case, however, as a study published in PLOS Neglected Tropical Diseases (now there’s a specific topic) has found. The researchers detail the glorious history of helminth infestation in the United Kingdom from the Victorian era all the way back to prehistory, scouring hundreds of skeletons found in 17 sites across the country for eggs, which can remain intact for thousands of years.
The researchers found that two eras in particular had very high rates of infection. Unsurprisingly, the late medieval era was one of them, but the other is less obvious. The Romans were famous for their hygiene, their baths, and their plumbing, but maybe they also should be famous for the abundance of worms in their bellies. That doesn’t make sense at first: Shouldn’t good hygiene lower infection? The benefits of a good sewer system, however, are lessened when the waste containing said infectious organisms is used to fertilize crops. Recycling is generally a good thing, but less so when you’re recycling parasitic worms.
Curiously, of the three sites from the industrial age, only the one in London had high levels of worm infestation. Considering how dirty and cramped 19th-century British cities were, one might expect disease to run rampant (tuberculosis certainly did), but the sites in Oxford and Birmingham were almost devoid of worms. The researchers theorized that this was because of access to clean well water. Or maybe worms just have a thing for London. [Editor’s note: It’s probably not that.]
Facial Follicular Spicules: A Rare Cutaneous Presentation of Trichodysplasia Spinulosa
To the Editor:
A 57-year-old man with hypertension, dyslipidemia, and congestive heart failure presented with a disfiguring eruption comprised of asymptomatic papules on the face that appeared 12 months post–heart transplantation. Immunosuppressive medications included mycophenolic acid and tacrolimus ointment (FK506). The pinpoint papules spread from the central face to the ears, arms, and legs. Physical examination revealed multiple 0.5- to 1-mm flesh-colored papules over the glabella, nose, nasolabial folds, philtrum, chin, ears, arms, and legs sparing the trunk. The initial appearance of the facial rash resembled the surface of a nutmeg grater with central white spiny excrescences overlying fine papules (spinulosism)(Figure 1). In addition, eyebrow alopecia was present.
A 3-mm punch biopsy of a papule with a central spine was performed on the left thigh. Microscopic examination revealed marked dilatation of anagen hair follicles with a proliferation of haphazard inner root sheath cells replacing the follicular lumen. Hair shafts were absent, and plugged infundibula were observed (Figure 2). The inner root sheath keratinocytes were enlarged and dystrophic with deeply eosinophilic trichohyalin granules (Figure 3). The epidermis, outer root sheath epithelium, and eccrine structures were unremarkable.
Transmission electron microscopy (TEM) confirmed the presence of intranuclear viral inclusions within affected inner root sheath keratinocytes composed of nonenveloped icosahedral viral particles measuring 33 to 38 nm in diameter (Figure 4). These findings morphologically were consistent with a polyomavirus. No intracytoplasmic or extracellular viral particles were identified. The clinical history, physical examination, histopathology, and electron microscopy features strongly supported the diagnosis of trichodysplasia spinulosa (TS) despite insufficient material being retrieved for polymerase chain reaction identification.
Trichodysplasia spinulosa was first described by Haycox et al1 in 1999. The authors suggested a viral etiology. Eleven years later, TS-associated polyomavirus (TSPyV) was identified by van der Meijden et al.2 Follicular keratinocytes are the specific target for TSPyV.3 Evidence has been presented suggesting that TS is caused by a primary infection or reactivation of TSPyV in the setting of immunosuppression.4,5
Patients with TS present with papular eruptions that appear on the central face with spiny excrescences and various degrees of alopecia involving the eyebrows or eyelashes. Histopathologic features include distended hair follicles with expansion of inner root sheath cells, eosinophilic trichohyalin granules, and the absence of hair shafts. The viral protein can be verified through immunohistochemistry TSPyV VP1 staining that demonstrates co-localization with trichohyalin. Viral particles also can be visualized as 35- to 38-nm intranuclear particles with an organized crystalloid morphology on TEM.6,7 The negative polymerase chain reaction in our patient could be the result of suboptimal template DNA concentration extracted from the limited amount of tissue remaining in the block after hematoxylin and eosin staining.
The clinical differential diagnosis of central facial spinulosism includes the follicular spicules of multiple myeloma (FSMM). In fact, FSMM and TS can only be differentiated after obtaining a blood profile and bone marrow biopsy that excludes the diagnosis of FSMM. A history of immunosuppression typically suggests TS. Histopathology often is equivocal in FSMM8; however, TEM reveals viral particles (TSPyV) in TS. Transmission electron microscopy in FSMM demonstrates fibrillary structures arranged in a paracrystalline configuration with unknown significance instead of viral particles. Despite the absence of viral particles on TEM, a low mean copy number of Merkel cell polyomavirus was isolated from a patient with FSMM who responded dramatically to treatment with topical cidofovir gel 1%.8 In addition to treating the underlying multiple myeloma in FSMM, topical cidofovir gel 1% also may have a role in treatment of these patients, suggesting a possible viral rather than simply paraneoplastic etiology of FSMM. Therefore, polyomavirus infection should be considered in the initial workup of any patient with fine facial follicular spicules.
The most effective management of TS in transplant recipients is to reduce immunosuppression to the lowest level possible without jeopardizing the transplanted organ.9 In our case, reduction of immunosuppressive drugs was not possible. In fact, immunosuppression in our patient was increased following evidence of early rejection of the heart transplant. Although manual extraction of the keratin spicules resulted in considerable improvement in a similar facial eruption in a patient with pediatric pre–B-cell acute lymphoblastic leukemia developing TS,10 it is impossible to apply this approach to patients such as ours who have thousands of tiny lesions. Fortunately, custom-compounded cidofovir gel 1% applied twice daily to the patient’s face and ears for 4 weeks led to near-complete clearance at follow-up (Figure 5). Due to the high cost of the medication (approaching $700 for one tube), our patient applied this medication to the face only several times weekly with excellent improvement. Thus, it appears that it is possible to suppress this virus with topical medication alone.
Polyomavirus infection should be considered in patients presenting with fine follicular spiny papules, especially those who are immunosuppressed. The possibility of coexisting multiple myeloma should be excluded.
Acknowledgment—We sincerely thank Glenn A. Hoskins (Jackson, Mississippi), the electron microscopy technologist, for the detection of viral particles and the electron microscope photographs.
- Haycox CL, Kim S, Fleckman P, et al. Trichodysplasia spinulosa: a newly described folliculocentric viral infection in an immunocompromised host. J Investig Dermatol Symp Proc. 1999;4:268-271.
- van der Meijden E, Janssens RWA, Lauber C, et al. Discovery of a new human polyomavirus associated with trichodysplasia spinulosa in an immunocompromized patient. PLoS Pathog. 2010;6:E1001024.
- Rouanet J, Aubin F, Gaboriaud P, et al. Trichodysplasia spinulosa: a polyomavirus infection specifically targeting follicular keratinocytes in immunocompromised patients. Br J Dermatol. 2016;174:629-632.
- van der Meijden E, Kazem S, Burgers MM, et al. Seroprevalence of trichodysplasia spinulosa-associated polyomavirus. Emerg Infect Dis. 2011;17:1355-1363.
- van der Meijden E, Horváth B, Nijland M, et al. Primary polyomavirus infection, not reactivation, as the cause of trichodysplasia spinulosa in immunocompromised patients. J Infect Dis. 2017;215:1080-1084.
- Fischer MK, Kao GF, Nguyen HP, et al. Specific detection of trichodysplasia spinulosa-associated polyomavirus DNA in skin and renal allograft tissues in a patient with trichodysplasia spinulosa. Arch Dermatol. 2012;148:726-733.
- Kazem S, van der Meijden E, Feltkamp MC. The trichodysplasia spinulosa-associated polyomavirus: virological background and clinical implications. APMIS. 2013;121:770-782.
- van Boheemen S, Jones T, Muhlemann B, et al. Cidofovir gel as treatment of follicular spicules in multiple myeloma. JAMA Dermatol. 2015;151:82-84.
- DeCrescenzo AJ, Philips RC, Wilkerson MG. Trichodysplasia spinulosa: a rare complication of immunosuppression. JAAD Case Rep. 2016;2:307-309.
- Barton M, Lockhart S, Sidbury R, et al. Trichodysplasia spinulosa in a 7-year-old boy managed using physical extraction of keratin spicules. Pediatr Dermatol. 2017;34:E74-E76.
To the Editor:
A 57-year-old man with hypertension, dyslipidemia, and congestive heart failure presented with a disfiguring eruption comprised of asymptomatic papules on the face that appeared 12 months post–heart transplantation. Immunosuppressive medications included mycophenolic acid and tacrolimus ointment (FK506). The pinpoint papules spread from the central face to the ears, arms, and legs. Physical examination revealed multiple 0.5- to 1-mm flesh-colored papules over the glabella, nose, nasolabial folds, philtrum, chin, ears, arms, and legs sparing the trunk. The initial appearance of the facial rash resembled the surface of a nutmeg grater with central white spiny excrescences overlying fine papules (spinulosism)(Figure 1). In addition, eyebrow alopecia was present.
A 3-mm punch biopsy of a papule with a central spine was performed on the left thigh. Microscopic examination revealed marked dilatation of anagen hair follicles with a proliferation of haphazard inner root sheath cells replacing the follicular lumen. Hair shafts were absent, and plugged infundibula were observed (Figure 2). The inner root sheath keratinocytes were enlarged and dystrophic with deeply eosinophilic trichohyalin granules (Figure 3). The epidermis, outer root sheath epithelium, and eccrine structures were unremarkable.
Transmission electron microscopy (TEM) confirmed the presence of intranuclear viral inclusions within affected inner root sheath keratinocytes composed of nonenveloped icosahedral viral particles measuring 33 to 38 nm in diameter (Figure 4). These findings morphologically were consistent with a polyomavirus. No intracytoplasmic or extracellular viral particles were identified. The clinical history, physical examination, histopathology, and electron microscopy features strongly supported the diagnosis of trichodysplasia spinulosa (TS) despite insufficient material being retrieved for polymerase chain reaction identification.
Trichodysplasia spinulosa was first described by Haycox et al1 in 1999. The authors suggested a viral etiology. Eleven years later, TS-associated polyomavirus (TSPyV) was identified by van der Meijden et al.2 Follicular keratinocytes are the specific target for TSPyV.3 Evidence has been presented suggesting that TS is caused by a primary infection or reactivation of TSPyV in the setting of immunosuppression.4,5
Patients with TS present with papular eruptions that appear on the central face with spiny excrescences and various degrees of alopecia involving the eyebrows or eyelashes. Histopathologic features include distended hair follicles with expansion of inner root sheath cells, eosinophilic trichohyalin granules, and the absence of hair shafts. The viral protein can be verified through immunohistochemistry TSPyV VP1 staining that demonstrates co-localization with trichohyalin. Viral particles also can be visualized as 35- to 38-nm intranuclear particles with an organized crystalloid morphology on TEM.6,7 The negative polymerase chain reaction in our patient could be the result of suboptimal template DNA concentration extracted from the limited amount of tissue remaining in the block after hematoxylin and eosin staining.
The clinical differential diagnosis of central facial spinulosism includes the follicular spicules of multiple myeloma (FSMM). In fact, FSMM and TS can only be differentiated after obtaining a blood profile and bone marrow biopsy that excludes the diagnosis of FSMM. A history of immunosuppression typically suggests TS. Histopathology often is equivocal in FSMM8; however, TEM reveals viral particles (TSPyV) in TS. Transmission electron microscopy in FSMM demonstrates fibrillary structures arranged in a paracrystalline configuration with unknown significance instead of viral particles. Despite the absence of viral particles on TEM, a low mean copy number of Merkel cell polyomavirus was isolated from a patient with FSMM who responded dramatically to treatment with topical cidofovir gel 1%.8 In addition to treating the underlying multiple myeloma in FSMM, topical cidofovir gel 1% also may have a role in treatment of these patients, suggesting a possible viral rather than simply paraneoplastic etiology of FSMM. Therefore, polyomavirus infection should be considered in the initial workup of any patient with fine facial follicular spicules.
The most effective management of TS in transplant recipients is to reduce immunosuppression to the lowest level possible without jeopardizing the transplanted organ.9 In our case, reduction of immunosuppressive drugs was not possible. In fact, immunosuppression in our patient was increased following evidence of early rejection of the heart transplant. Although manual extraction of the keratin spicules resulted in considerable improvement in a similar facial eruption in a patient with pediatric pre–B-cell acute lymphoblastic leukemia developing TS,10 it is impossible to apply this approach to patients such as ours who have thousands of tiny lesions. Fortunately, custom-compounded cidofovir gel 1% applied twice daily to the patient’s face and ears for 4 weeks led to near-complete clearance at follow-up (Figure 5). Due to the high cost of the medication (approaching $700 for one tube), our patient applied this medication to the face only several times weekly with excellent improvement. Thus, it appears that it is possible to suppress this virus with topical medication alone.
Polyomavirus infection should be considered in patients presenting with fine follicular spiny papules, especially those who are immunosuppressed. The possibility of coexisting multiple myeloma should be excluded.
Acknowledgment—We sincerely thank Glenn A. Hoskins (Jackson, Mississippi), the electron microscopy technologist, for the detection of viral particles and the electron microscope photographs.
To the Editor:
A 57-year-old man with hypertension, dyslipidemia, and congestive heart failure presented with a disfiguring eruption comprised of asymptomatic papules on the face that appeared 12 months post–heart transplantation. Immunosuppressive medications included mycophenolic acid and tacrolimus ointment (FK506). The pinpoint papules spread from the central face to the ears, arms, and legs. Physical examination revealed multiple 0.5- to 1-mm flesh-colored papules over the glabella, nose, nasolabial folds, philtrum, chin, ears, arms, and legs sparing the trunk. The initial appearance of the facial rash resembled the surface of a nutmeg grater with central white spiny excrescences overlying fine papules (spinulosism)(Figure 1). In addition, eyebrow alopecia was present.
A 3-mm punch biopsy of a papule with a central spine was performed on the left thigh. Microscopic examination revealed marked dilatation of anagen hair follicles with a proliferation of haphazard inner root sheath cells replacing the follicular lumen. Hair shafts were absent, and plugged infundibula were observed (Figure 2). The inner root sheath keratinocytes were enlarged and dystrophic with deeply eosinophilic trichohyalin granules (Figure 3). The epidermis, outer root sheath epithelium, and eccrine structures were unremarkable.
Transmission electron microscopy (TEM) confirmed the presence of intranuclear viral inclusions within affected inner root sheath keratinocytes composed of nonenveloped icosahedral viral particles measuring 33 to 38 nm in diameter (Figure 4). These findings morphologically were consistent with a polyomavirus. No intracytoplasmic or extracellular viral particles were identified. The clinical history, physical examination, histopathology, and electron microscopy features strongly supported the diagnosis of trichodysplasia spinulosa (TS) despite insufficient material being retrieved for polymerase chain reaction identification.
Trichodysplasia spinulosa was first described by Haycox et al1 in 1999. The authors suggested a viral etiology. Eleven years later, TS-associated polyomavirus (TSPyV) was identified by van der Meijden et al.2 Follicular keratinocytes are the specific target for TSPyV.3 Evidence has been presented suggesting that TS is caused by a primary infection or reactivation of TSPyV in the setting of immunosuppression.4,5
Patients with TS present with papular eruptions that appear on the central face with spiny excrescences and various degrees of alopecia involving the eyebrows or eyelashes. Histopathologic features include distended hair follicles with expansion of inner root sheath cells, eosinophilic trichohyalin granules, and the absence of hair shafts. The viral protein can be verified through immunohistochemistry TSPyV VP1 staining that demonstrates co-localization with trichohyalin. Viral particles also can be visualized as 35- to 38-nm intranuclear particles with an organized crystalloid morphology on TEM.6,7 The negative polymerase chain reaction in our patient could be the result of suboptimal template DNA concentration extracted from the limited amount of tissue remaining in the block after hematoxylin and eosin staining.
The clinical differential diagnosis of central facial spinulosism includes the follicular spicules of multiple myeloma (FSMM). In fact, FSMM and TS can only be differentiated after obtaining a blood profile and bone marrow biopsy that excludes the diagnosis of FSMM. A history of immunosuppression typically suggests TS. Histopathology often is equivocal in FSMM8; however, TEM reveals viral particles (TSPyV) in TS. Transmission electron microscopy in FSMM demonstrates fibrillary structures arranged in a paracrystalline configuration with unknown significance instead of viral particles. Despite the absence of viral particles on TEM, a low mean copy number of Merkel cell polyomavirus was isolated from a patient with FSMM who responded dramatically to treatment with topical cidofovir gel 1%.8 In addition to treating the underlying multiple myeloma in FSMM, topical cidofovir gel 1% also may have a role in treatment of these patients, suggesting a possible viral rather than simply paraneoplastic etiology of FSMM. Therefore, polyomavirus infection should be considered in the initial workup of any patient with fine facial follicular spicules.
The most effective management of TS in transplant recipients is to reduce immunosuppression to the lowest level possible without jeopardizing the transplanted organ.9 In our case, reduction of immunosuppressive drugs was not possible. In fact, immunosuppression in our patient was increased following evidence of early rejection of the heart transplant. Although manual extraction of the keratin spicules resulted in considerable improvement in a similar facial eruption in a patient with pediatric pre–B-cell acute lymphoblastic leukemia developing TS,10 it is impossible to apply this approach to patients such as ours who have thousands of tiny lesions. Fortunately, custom-compounded cidofovir gel 1% applied twice daily to the patient’s face and ears for 4 weeks led to near-complete clearance at follow-up (Figure 5). Due to the high cost of the medication (approaching $700 for one tube), our patient applied this medication to the face only several times weekly with excellent improvement. Thus, it appears that it is possible to suppress this virus with topical medication alone.
Polyomavirus infection should be considered in patients presenting with fine follicular spiny papules, especially those who are immunosuppressed. The possibility of coexisting multiple myeloma should be excluded.
Acknowledgment—We sincerely thank Glenn A. Hoskins (Jackson, Mississippi), the electron microscopy technologist, for the detection of viral particles and the electron microscope photographs.
- Haycox CL, Kim S, Fleckman P, et al. Trichodysplasia spinulosa: a newly described folliculocentric viral infection in an immunocompromised host. J Investig Dermatol Symp Proc. 1999;4:268-271.
- van der Meijden E, Janssens RWA, Lauber C, et al. Discovery of a new human polyomavirus associated with trichodysplasia spinulosa in an immunocompromized patient. PLoS Pathog. 2010;6:E1001024.
- Rouanet J, Aubin F, Gaboriaud P, et al. Trichodysplasia spinulosa: a polyomavirus infection specifically targeting follicular keratinocytes in immunocompromised patients. Br J Dermatol. 2016;174:629-632.
- van der Meijden E, Kazem S, Burgers MM, et al. Seroprevalence of trichodysplasia spinulosa-associated polyomavirus. Emerg Infect Dis. 2011;17:1355-1363.
- van der Meijden E, Horváth B, Nijland M, et al. Primary polyomavirus infection, not reactivation, as the cause of trichodysplasia spinulosa in immunocompromised patients. J Infect Dis. 2017;215:1080-1084.
- Fischer MK, Kao GF, Nguyen HP, et al. Specific detection of trichodysplasia spinulosa-associated polyomavirus DNA in skin and renal allograft tissues in a patient with trichodysplasia spinulosa. Arch Dermatol. 2012;148:726-733.
- Kazem S, van der Meijden E, Feltkamp MC. The trichodysplasia spinulosa-associated polyomavirus: virological background and clinical implications. APMIS. 2013;121:770-782.
- van Boheemen S, Jones T, Muhlemann B, et al. Cidofovir gel as treatment of follicular spicules in multiple myeloma. JAMA Dermatol. 2015;151:82-84.
- DeCrescenzo AJ, Philips RC, Wilkerson MG. Trichodysplasia spinulosa: a rare complication of immunosuppression. JAAD Case Rep. 2016;2:307-309.
- Barton M, Lockhart S, Sidbury R, et al. Trichodysplasia spinulosa in a 7-year-old boy managed using physical extraction of keratin spicules. Pediatr Dermatol. 2017;34:E74-E76.
- Haycox CL, Kim S, Fleckman P, et al. Trichodysplasia spinulosa: a newly described folliculocentric viral infection in an immunocompromised host. J Investig Dermatol Symp Proc. 1999;4:268-271.
- van der Meijden E, Janssens RWA, Lauber C, et al. Discovery of a new human polyomavirus associated with trichodysplasia spinulosa in an immunocompromized patient. PLoS Pathog. 2010;6:E1001024.
- Rouanet J, Aubin F, Gaboriaud P, et al. Trichodysplasia spinulosa: a polyomavirus infection specifically targeting follicular keratinocytes in immunocompromised patients. Br J Dermatol. 2016;174:629-632.
- van der Meijden E, Kazem S, Burgers MM, et al. Seroprevalence of trichodysplasia spinulosa-associated polyomavirus. Emerg Infect Dis. 2011;17:1355-1363.
- van der Meijden E, Horváth B, Nijland M, et al. Primary polyomavirus infection, not reactivation, as the cause of trichodysplasia spinulosa in immunocompromised patients. J Infect Dis. 2017;215:1080-1084.
- Fischer MK, Kao GF, Nguyen HP, et al. Specific detection of trichodysplasia spinulosa-associated polyomavirus DNA in skin and renal allograft tissues in a patient with trichodysplasia spinulosa. Arch Dermatol. 2012;148:726-733.
- Kazem S, van der Meijden E, Feltkamp MC. The trichodysplasia spinulosa-associated polyomavirus: virological background and clinical implications. APMIS. 2013;121:770-782.
- van Boheemen S, Jones T, Muhlemann B, et al. Cidofovir gel as treatment of follicular spicules in multiple myeloma. JAMA Dermatol. 2015;151:82-84.
- DeCrescenzo AJ, Philips RC, Wilkerson MG. Trichodysplasia spinulosa: a rare complication of immunosuppression. JAAD Case Rep. 2016;2:307-309.
- Barton M, Lockhart S, Sidbury R, et al. Trichodysplasia spinulosa in a 7-year-old boy managed using physical extraction of keratin spicules. Pediatr Dermatol. 2017;34:E74-E76.
Practice Points
- Trichodysplasia spinulosa (TS) is a rare skin disease caused by primary TS-associated polyomavirus (TSPyV) infecting follicular keratinocytes in immunocompromised patients.
- Trichodysplasia spinulosa typically presents with papular eruptions that appear on the central face with spiny excrescences and various degrees of alopecia involving the eyebrows or eyelashes.
- The viral protein can be verified through immunohistochemistry TSPyV major capsid protein VP1 staining or can be visualized on transmission electron microscopy.
- Follicular spicules of multiple myeloma should be ruled out before initiating treatment with cidofovir gel 1% for TS.
NB-UVB phototherapy plays a key role in psoriasis treatment, expert says
BOSTON – In 2012, about 50% of patients receiving phototherapy at Brigham and Women’s Hospital in Boston were being treated for psoriasis. A decade later, that proportion has dropped to 20%.
Several factors have contributed to this trend, namely, the development of biologics, the COVID-19 pandemic, “and the rise of home phototherapy options,” Elizabeth A. Buzney, MD, codirector of the phototherapy center at Brigham and Women’s department of dermatology, said at the annual meeting of the American Academy of Dermatology. In her clinical opinion, phototherapy plays an essential role in the treatment of psoriasis.
“It is medically and financially responsible to review the option of phototherapy with every psoriasis patient,” Dr. Buzney said. “Many patients are not medical or financial candidates for biologic/apremilast therapy, or just would prefer nonsystemic therapy.”
In one meta-analysis, the proportion of patients achieving Psoriasis Area and Severity Index (PASI) 75 with NB-UVB therapy was 70% after 20-40 sessions, just below the efficacy of newer biologics – but better than ustekinumab and adalimumab.
“Phototherapy is not so far out of range as you might think it is,” she said, noting that other studies of NB-UVB therapy show PASI 75 responses of 62% and PASI 90 responses of 40%.
Phototherapy can also be an appealing option because biologics aren’t the best option for all patients with psoriasis. They are expensive for the health care system and potentially for patients, require initial and potentially continued lab testing and monitoring, and require injections, “which some patients don’t like,” said Dr. Buzney, who is also vice-chair of clinical affairs at the Brigham and Women’s Hospital department of dermatology. “There’s an infrequent risk of serious infection and there is risk in patients with HIV, TB, and hepatitis that you have to address. There is also concern for the impact of biologics on patients with a recent cancer.”
On the other hand, few contraindications to NB-UVB exist. According to joint American Academy of Dermatology-National Psoriasis Foundation guidelines on the management and treatment of psoriasis with phototherapy, published in 2019, NB-UVB therapy is only contraindicated in patients with xeroderma pigmentosa and other photosensitive disorders. Concurrent use of cyclosporine and NB-UVB treatment is also contraindicated because of the calculated increase in risk of skin cancer, extrapolated from data on risk with cyclosporine and PUVA (psoralen and ultraviolet A therapy).
The guidelines state that NB-UVB can be used with caution in lupus patients with no history of photosensitivity and who are SS-A negative, as well as patients with a history of melanoma or multiple nonmelanoma skin cancers, a history of recurrent oral herpes simplex virus infection, a history of arsenic intake, prior exposure to ionizing radiation, and those taking photosensitizing medications (since NB-UVB lamps emit “negligible” UVA).
It’s also safe to use during pregnancy and in children. “It’s safe and effective for the right patient,” Dr. Buzney said, discussing how phototherapy can be modified to accommodate children. “You can consider a slower dose-increased regimen. Will children keep the eye protection on? That’s a tricky one. How are you going to manage their anxiety during treatment and involve their family?”
Subgroups of patients who demonstrate a better response to NB-UVB treatment include those with guttate psoriasis, compared with plaque psoriasis, nonsmokers, those with a lower BMI, those with a higher baseline PASI, and those who demonstrate a faster trajectory of clinical response over the first 2-3 weeks of treatment.
Why would one not use phototherapy for psoriasis? “Cost and convenience,” Dr. Buzney said. “There is lost time/revenue to commute to treatment, which may involve multiple times per week. Coming to a public space when COVID-19 is still lingering is another concern, as are the out-of-pocket costs for copays and parking.”
For these reasons, she considers home phototherapy as a transformative option for many patients. Home phototherapy booths provide a safe and effective way to use NB-UVB phototherapy while minimizing copays and commuting costs. The one-time price tag of home NB-UVB booths runs between $5,000 and $7,000, but that is “much less expensive than the biologics,” which can cost $40,000-$50,000 per year, she said.
A small cross-sectional study of office- versus home-based NB-UVB in patients with vitiligo found a cost savings for home-based NB-UVB after 3 months.
One of the challenges with home phototherapy is the lack of long-term studies on patient use. In a small study Dr. Buzney conducted of 30 patients who were prescribed home phototherapy in the last 5 years, 65% practiced (or had practiced) conservative dosing, 83% had continued care with a dermatologist, 19% reported sunburns (5 mild and 1 severe), and 50% had discontinued the therapy at the time of survey because of a perceived lack of efficacy and inconvenience. But 30% of those who had stopped had done so within one month of getting their home booth.
“This tells me that we have to educate our patients better about what expectations should be and make sure they understand how to use their booths,” she said. “Home phototherapy has changed my practice, but not everyone is a candidate for it. Some patients are not dependable. Others are unable to understand instructions.”
Cost to purchase a NB-UVB booth is also an issue, she noted. “Typically, a percentage of cost is covered by insurance, but it’s problematic to purchase a booth if patients don’t know it’s going to work for them or not. Then you have college students who don’t have the space in their apartment or dorm room for a booth.”
Dr. Buzney reported having no relevant financial conflicts.
BOSTON – In 2012, about 50% of patients receiving phototherapy at Brigham and Women’s Hospital in Boston were being treated for psoriasis. A decade later, that proportion has dropped to 20%.
Several factors have contributed to this trend, namely, the development of biologics, the COVID-19 pandemic, “and the rise of home phototherapy options,” Elizabeth A. Buzney, MD, codirector of the phototherapy center at Brigham and Women’s department of dermatology, said at the annual meeting of the American Academy of Dermatology. In her clinical opinion, phototherapy plays an essential role in the treatment of psoriasis.
“It is medically and financially responsible to review the option of phototherapy with every psoriasis patient,” Dr. Buzney said. “Many patients are not medical or financial candidates for biologic/apremilast therapy, or just would prefer nonsystemic therapy.”
In one meta-analysis, the proportion of patients achieving Psoriasis Area and Severity Index (PASI) 75 with NB-UVB therapy was 70% after 20-40 sessions, just below the efficacy of newer biologics – but better than ustekinumab and adalimumab.
“Phototherapy is not so far out of range as you might think it is,” she said, noting that other studies of NB-UVB therapy show PASI 75 responses of 62% and PASI 90 responses of 40%.
Phototherapy can also be an appealing option because biologics aren’t the best option for all patients with psoriasis. They are expensive for the health care system and potentially for patients, require initial and potentially continued lab testing and monitoring, and require injections, “which some patients don’t like,” said Dr. Buzney, who is also vice-chair of clinical affairs at the Brigham and Women’s Hospital department of dermatology. “There’s an infrequent risk of serious infection and there is risk in patients with HIV, TB, and hepatitis that you have to address. There is also concern for the impact of biologics on patients with a recent cancer.”
On the other hand, few contraindications to NB-UVB exist. According to joint American Academy of Dermatology-National Psoriasis Foundation guidelines on the management and treatment of psoriasis with phototherapy, published in 2019, NB-UVB therapy is only contraindicated in patients with xeroderma pigmentosa and other photosensitive disorders. Concurrent use of cyclosporine and NB-UVB treatment is also contraindicated because of the calculated increase in risk of skin cancer, extrapolated from data on risk with cyclosporine and PUVA (psoralen and ultraviolet A therapy).
The guidelines state that NB-UVB can be used with caution in lupus patients with no history of photosensitivity and who are SS-A negative, as well as patients with a history of melanoma or multiple nonmelanoma skin cancers, a history of recurrent oral herpes simplex virus infection, a history of arsenic intake, prior exposure to ionizing radiation, and those taking photosensitizing medications (since NB-UVB lamps emit “negligible” UVA).
It’s also safe to use during pregnancy and in children. “It’s safe and effective for the right patient,” Dr. Buzney said, discussing how phototherapy can be modified to accommodate children. “You can consider a slower dose-increased regimen. Will children keep the eye protection on? That’s a tricky one. How are you going to manage their anxiety during treatment and involve their family?”
Subgroups of patients who demonstrate a better response to NB-UVB treatment include those with guttate psoriasis, compared with plaque psoriasis, nonsmokers, those with a lower BMI, those with a higher baseline PASI, and those who demonstrate a faster trajectory of clinical response over the first 2-3 weeks of treatment.
Why would one not use phototherapy for psoriasis? “Cost and convenience,” Dr. Buzney said. “There is lost time/revenue to commute to treatment, which may involve multiple times per week. Coming to a public space when COVID-19 is still lingering is another concern, as are the out-of-pocket costs for copays and parking.”
For these reasons, she considers home phototherapy as a transformative option for many patients. Home phototherapy booths provide a safe and effective way to use NB-UVB phototherapy while minimizing copays and commuting costs. The one-time price tag of home NB-UVB booths runs between $5,000 and $7,000, but that is “much less expensive than the biologics,” which can cost $40,000-$50,000 per year, she said.
A small cross-sectional study of office- versus home-based NB-UVB in patients with vitiligo found a cost savings for home-based NB-UVB after 3 months.
One of the challenges with home phototherapy is the lack of long-term studies on patient use. In a small study Dr. Buzney conducted of 30 patients who were prescribed home phototherapy in the last 5 years, 65% practiced (or had practiced) conservative dosing, 83% had continued care with a dermatologist, 19% reported sunburns (5 mild and 1 severe), and 50% had discontinued the therapy at the time of survey because of a perceived lack of efficacy and inconvenience. But 30% of those who had stopped had done so within one month of getting their home booth.
“This tells me that we have to educate our patients better about what expectations should be and make sure they understand how to use their booths,” she said. “Home phototherapy has changed my practice, but not everyone is a candidate for it. Some patients are not dependable. Others are unable to understand instructions.”
Cost to purchase a NB-UVB booth is also an issue, she noted. “Typically, a percentage of cost is covered by insurance, but it’s problematic to purchase a booth if patients don’t know it’s going to work for them or not. Then you have college students who don’t have the space in their apartment or dorm room for a booth.”
Dr. Buzney reported having no relevant financial conflicts.
BOSTON – In 2012, about 50% of patients receiving phototherapy at Brigham and Women’s Hospital in Boston were being treated for psoriasis. A decade later, that proportion has dropped to 20%.
Several factors have contributed to this trend, namely, the development of biologics, the COVID-19 pandemic, “and the rise of home phototherapy options,” Elizabeth A. Buzney, MD, codirector of the phototherapy center at Brigham and Women’s department of dermatology, said at the annual meeting of the American Academy of Dermatology. In her clinical opinion, phototherapy plays an essential role in the treatment of psoriasis.
“It is medically and financially responsible to review the option of phototherapy with every psoriasis patient,” Dr. Buzney said. “Many patients are not medical or financial candidates for biologic/apremilast therapy, or just would prefer nonsystemic therapy.”
In one meta-analysis, the proportion of patients achieving Psoriasis Area and Severity Index (PASI) 75 with NB-UVB therapy was 70% after 20-40 sessions, just below the efficacy of newer biologics – but better than ustekinumab and adalimumab.
“Phototherapy is not so far out of range as you might think it is,” she said, noting that other studies of NB-UVB therapy show PASI 75 responses of 62% and PASI 90 responses of 40%.
Phototherapy can also be an appealing option because biologics aren’t the best option for all patients with psoriasis. They are expensive for the health care system and potentially for patients, require initial and potentially continued lab testing and monitoring, and require injections, “which some patients don’t like,” said Dr. Buzney, who is also vice-chair of clinical affairs at the Brigham and Women’s Hospital department of dermatology. “There’s an infrequent risk of serious infection and there is risk in patients with HIV, TB, and hepatitis that you have to address. There is also concern for the impact of biologics on patients with a recent cancer.”
On the other hand, few contraindications to NB-UVB exist. According to joint American Academy of Dermatology-National Psoriasis Foundation guidelines on the management and treatment of psoriasis with phototherapy, published in 2019, NB-UVB therapy is only contraindicated in patients with xeroderma pigmentosa and other photosensitive disorders. Concurrent use of cyclosporine and NB-UVB treatment is also contraindicated because of the calculated increase in risk of skin cancer, extrapolated from data on risk with cyclosporine and PUVA (psoralen and ultraviolet A therapy).
The guidelines state that NB-UVB can be used with caution in lupus patients with no history of photosensitivity and who are SS-A negative, as well as patients with a history of melanoma or multiple nonmelanoma skin cancers, a history of recurrent oral herpes simplex virus infection, a history of arsenic intake, prior exposure to ionizing radiation, and those taking photosensitizing medications (since NB-UVB lamps emit “negligible” UVA).
It’s also safe to use during pregnancy and in children. “It’s safe and effective for the right patient,” Dr. Buzney said, discussing how phototherapy can be modified to accommodate children. “You can consider a slower dose-increased regimen. Will children keep the eye protection on? That’s a tricky one. How are you going to manage their anxiety during treatment and involve their family?”
Subgroups of patients who demonstrate a better response to NB-UVB treatment include those with guttate psoriasis, compared with plaque psoriasis, nonsmokers, those with a lower BMI, those with a higher baseline PASI, and those who demonstrate a faster trajectory of clinical response over the first 2-3 weeks of treatment.
Why would one not use phototherapy for psoriasis? “Cost and convenience,” Dr. Buzney said. “There is lost time/revenue to commute to treatment, which may involve multiple times per week. Coming to a public space when COVID-19 is still lingering is another concern, as are the out-of-pocket costs for copays and parking.”
For these reasons, she considers home phototherapy as a transformative option for many patients. Home phototherapy booths provide a safe and effective way to use NB-UVB phototherapy while minimizing copays and commuting costs. The one-time price tag of home NB-UVB booths runs between $5,000 and $7,000, but that is “much less expensive than the biologics,” which can cost $40,000-$50,000 per year, she said.
A small cross-sectional study of office- versus home-based NB-UVB in patients with vitiligo found a cost savings for home-based NB-UVB after 3 months.
One of the challenges with home phototherapy is the lack of long-term studies on patient use. In a small study Dr. Buzney conducted of 30 patients who were prescribed home phototherapy in the last 5 years, 65% practiced (or had practiced) conservative dosing, 83% had continued care with a dermatologist, 19% reported sunburns (5 mild and 1 severe), and 50% had discontinued the therapy at the time of survey because of a perceived lack of efficacy and inconvenience. But 30% of those who had stopped had done so within one month of getting their home booth.
“This tells me that we have to educate our patients better about what expectations should be and make sure they understand how to use their booths,” she said. “Home phototherapy has changed my practice, but not everyone is a candidate for it. Some patients are not dependable. Others are unable to understand instructions.”
Cost to purchase a NB-UVB booth is also an issue, she noted. “Typically, a percentage of cost is covered by insurance, but it’s problematic to purchase a booth if patients don’t know it’s going to work for them or not. Then you have college students who don’t have the space in their apartment or dorm room for a booth.”
Dr. Buzney reported having no relevant financial conflicts.
AT AAD 22
Multimodal imaging of DIP-joint and SEC can help distinguish PsA from psoriasis or OA
Key clinical point: Ultrasound (US), magnetic resonance imaging (MRI), and X-ray could differentiate psoriatic arthritis (PsA) from psoriasis and hand osteoarthritis (OA) based on the degree of structural involvement in the distal interphalangeal (DIP)-joint and synovio-entheseal complex (SEC).
Major finding: US-detected new bone formation (NBF; risk ratio [RR] 0.52; P < .001) and DIP-joint synovial hypertrophy (RR 0.66; P = .005) along with PsA MRI scores (all P < .001) were associated with a lower risk for PsA vs. OA. Patients with PsA vs. psoriasis had a higher prevalence of X-ray entheseal change (mean difference 0.42; P = .024) and a higher trend toward US-detected NBF and erosions.
Study details: This prospective, cross-sectional study included 50 patients with DIP-joint PsA and nail involvement, 12 patients with psoriasis and nail involvement, and 13 patients with erosive and nonerosive OA.
Disclosures: The study was funded by Novartis, The Oak Foundation, and others. Some authors declared receiving speaker/consulting fees, research grants, or honoraria or serving as members of advisory board or consultants for several sources.
Source: Guldberg-Møller J et al. Multimodal imaging of the distal interphalangeal-joint synovio-entheseal complex in psoriatic arthritis (MIDAS): A cross-sectional study on the diagnostic accuracy of different imaging modalities comparing psoriatic arthritis to psoriasis and osteoarthritis. RMD Open. 2022;8:e002109 (Mar 28). Doi: 10.1136/rmdopen-2021-002109
Key clinical point: Ultrasound (US), magnetic resonance imaging (MRI), and X-ray could differentiate psoriatic arthritis (PsA) from psoriasis and hand osteoarthritis (OA) based on the degree of structural involvement in the distal interphalangeal (DIP)-joint and synovio-entheseal complex (SEC).
Major finding: US-detected new bone formation (NBF; risk ratio [RR] 0.52; P < .001) and DIP-joint synovial hypertrophy (RR 0.66; P = .005) along with PsA MRI scores (all P < .001) were associated with a lower risk for PsA vs. OA. Patients with PsA vs. psoriasis had a higher prevalence of X-ray entheseal change (mean difference 0.42; P = .024) and a higher trend toward US-detected NBF and erosions.
Study details: This prospective, cross-sectional study included 50 patients with DIP-joint PsA and nail involvement, 12 patients with psoriasis and nail involvement, and 13 patients with erosive and nonerosive OA.
Disclosures: The study was funded by Novartis, The Oak Foundation, and others. Some authors declared receiving speaker/consulting fees, research grants, or honoraria or serving as members of advisory board or consultants for several sources.
Source: Guldberg-Møller J et al. Multimodal imaging of the distal interphalangeal-joint synovio-entheseal complex in psoriatic arthritis (MIDAS): A cross-sectional study on the diagnostic accuracy of different imaging modalities comparing psoriatic arthritis to psoriasis and osteoarthritis. RMD Open. 2022;8:e002109 (Mar 28). Doi: 10.1136/rmdopen-2021-002109
Key clinical point: Ultrasound (US), magnetic resonance imaging (MRI), and X-ray could differentiate psoriatic arthritis (PsA) from psoriasis and hand osteoarthritis (OA) based on the degree of structural involvement in the distal interphalangeal (DIP)-joint and synovio-entheseal complex (SEC).
Major finding: US-detected new bone formation (NBF; risk ratio [RR] 0.52; P < .001) and DIP-joint synovial hypertrophy (RR 0.66; P = .005) along with PsA MRI scores (all P < .001) were associated with a lower risk for PsA vs. OA. Patients with PsA vs. psoriasis had a higher prevalence of X-ray entheseal change (mean difference 0.42; P = .024) and a higher trend toward US-detected NBF and erosions.
Study details: This prospective, cross-sectional study included 50 patients with DIP-joint PsA and nail involvement, 12 patients with psoriasis and nail involvement, and 13 patients with erosive and nonerosive OA.
Disclosures: The study was funded by Novartis, The Oak Foundation, and others. Some authors declared receiving speaker/consulting fees, research grants, or honoraria or serving as members of advisory board or consultants for several sources.
Source: Guldberg-Møller J et al. Multimodal imaging of the distal interphalangeal-joint synovio-entheseal complex in psoriatic arthritis (MIDAS): A cross-sectional study on the diagnostic accuracy of different imaging modalities comparing psoriatic arthritis to psoriasis and osteoarthritis. RMD Open. 2022;8:e002109 (Mar 28). Doi: 10.1136/rmdopen-2021-002109
Topical treatment for EB recommended for approval in the EU
A topical
(EMA’s) Committee for Medicinal Products for Human Use.“The benefit of Filsuvez is its ability to promote healing of EB partial thickness wounds,” the EMA said in an announcement on April 22. “It is thought to work by modulating inflammatory mediators and stimulating keratinocyte differentiation and migration, thereby promoting wound health and closure,” the statement adds.
The recommended indication for the product – developed by Amryt Pharmaceuticals DAC and currently designated as an orphan drug – is for the treatment of partial-thickness wounds associated with dystrophic and junctional EB in patients aged 6 months and older. The recommendation for approval came after the EMA sought and received external advice from independent physicians treating EB and from patients with the rare disease.
The most common side effects, according to the EMA announcement, are wound complications, application site reactions, wound infections, pruritus, and hypersensitivity reactions.
In February 2022, the Food and Drug Administration declined to approve the company’s new drug application as it was presented and asked the company to submit additional evidence of effectiveness for Oleogel-S10 in EB, the company announced at that time. The statement noted that the company was committed to working with the FDA to identify "the most expeditious pathway towards a potential approval.”
The company’s pivotal phase 3 trial enrolled 223 patients with EB, including 156 pediatric patients. The patients variously had three types of EB. The trial has two components: A 3-month, double-blind, randomized controlled phase, which has been completed, and an ongoing 24-month open-label, single-arm phase. The trial is being performed at 58 sites in 28 countries.
Results from the randomized controlled phase, reported in 2020, include a statistically significant increase in the proportion of patients achieving complete closure of an EB target wound within 45 days: 41.3% in the Oleogel-S10 group and 28.9% in the control group (P = .013). (Target wounds measured 10 cm² to 50 cm² and were present for at least 21 days but less than 9 months.) The safety profile of the treatment gel was acceptable and was well tolerated, compared with the control gel, according to Amryt’s press release. The results were presented at the European Academy of Dermatology and Venereology Congress in October 2020.
Data from a 12-month interim analysis of the follow-up phase were presented at the annual meeting of the American Academy of Dermatology in March 2022. Results showed further reductions in total body surface area percentage wounding to 5.4% among (from 7.4% at the end of the double-blind period and 12.1% at the beginning of the study) among the patients who continued treatment and who underwent assessment, according to a company press release. Treatment was well tolerated, and no new safety signals were identified, the release said.
A decision by the European Commission is expected within the next 2 months.
A version of this article first appeared on Medscape.com.
A topical
(EMA’s) Committee for Medicinal Products for Human Use.“The benefit of Filsuvez is its ability to promote healing of EB partial thickness wounds,” the EMA said in an announcement on April 22. “It is thought to work by modulating inflammatory mediators and stimulating keratinocyte differentiation and migration, thereby promoting wound health and closure,” the statement adds.
The recommended indication for the product – developed by Amryt Pharmaceuticals DAC and currently designated as an orphan drug – is for the treatment of partial-thickness wounds associated with dystrophic and junctional EB in patients aged 6 months and older. The recommendation for approval came after the EMA sought and received external advice from independent physicians treating EB and from patients with the rare disease.
The most common side effects, according to the EMA announcement, are wound complications, application site reactions, wound infections, pruritus, and hypersensitivity reactions.
In February 2022, the Food and Drug Administration declined to approve the company’s new drug application as it was presented and asked the company to submit additional evidence of effectiveness for Oleogel-S10 in EB, the company announced at that time. The statement noted that the company was committed to working with the FDA to identify "the most expeditious pathway towards a potential approval.”
The company’s pivotal phase 3 trial enrolled 223 patients with EB, including 156 pediatric patients. The patients variously had three types of EB. The trial has two components: A 3-month, double-blind, randomized controlled phase, which has been completed, and an ongoing 24-month open-label, single-arm phase. The trial is being performed at 58 sites in 28 countries.
Results from the randomized controlled phase, reported in 2020, include a statistically significant increase in the proportion of patients achieving complete closure of an EB target wound within 45 days: 41.3% in the Oleogel-S10 group and 28.9% in the control group (P = .013). (Target wounds measured 10 cm² to 50 cm² and were present for at least 21 days but less than 9 months.) The safety profile of the treatment gel was acceptable and was well tolerated, compared with the control gel, according to Amryt’s press release. The results were presented at the European Academy of Dermatology and Venereology Congress in October 2020.
Data from a 12-month interim analysis of the follow-up phase were presented at the annual meeting of the American Academy of Dermatology in March 2022. Results showed further reductions in total body surface area percentage wounding to 5.4% among (from 7.4% at the end of the double-blind period and 12.1% at the beginning of the study) among the patients who continued treatment and who underwent assessment, according to a company press release. Treatment was well tolerated, and no new safety signals were identified, the release said.
A decision by the European Commission is expected within the next 2 months.
A version of this article first appeared on Medscape.com.
A topical
(EMA’s) Committee for Medicinal Products for Human Use.“The benefit of Filsuvez is its ability to promote healing of EB partial thickness wounds,” the EMA said in an announcement on April 22. “It is thought to work by modulating inflammatory mediators and stimulating keratinocyte differentiation and migration, thereby promoting wound health and closure,” the statement adds.
The recommended indication for the product – developed by Amryt Pharmaceuticals DAC and currently designated as an orphan drug – is for the treatment of partial-thickness wounds associated with dystrophic and junctional EB in patients aged 6 months and older. The recommendation for approval came after the EMA sought and received external advice from independent physicians treating EB and from patients with the rare disease.
The most common side effects, according to the EMA announcement, are wound complications, application site reactions, wound infections, pruritus, and hypersensitivity reactions.
In February 2022, the Food and Drug Administration declined to approve the company’s new drug application as it was presented and asked the company to submit additional evidence of effectiveness for Oleogel-S10 in EB, the company announced at that time. The statement noted that the company was committed to working with the FDA to identify "the most expeditious pathway towards a potential approval.”
The company’s pivotal phase 3 trial enrolled 223 patients with EB, including 156 pediatric patients. The patients variously had three types of EB. The trial has two components: A 3-month, double-blind, randomized controlled phase, which has been completed, and an ongoing 24-month open-label, single-arm phase. The trial is being performed at 58 sites in 28 countries.
Results from the randomized controlled phase, reported in 2020, include a statistically significant increase in the proportion of patients achieving complete closure of an EB target wound within 45 days: 41.3% in the Oleogel-S10 group and 28.9% in the control group (P = .013). (Target wounds measured 10 cm² to 50 cm² and were present for at least 21 days but less than 9 months.) The safety profile of the treatment gel was acceptable and was well tolerated, compared with the control gel, according to Amryt’s press release. The results were presented at the European Academy of Dermatology and Venereology Congress in October 2020.
Data from a 12-month interim analysis of the follow-up phase were presented at the annual meeting of the American Academy of Dermatology in March 2022. Results showed further reductions in total body surface area percentage wounding to 5.4% among (from 7.4% at the end of the double-blind period and 12.1% at the beginning of the study) among the patients who continued treatment and who underwent assessment, according to a company press release. Treatment was well tolerated, and no new safety signals were identified, the release said.
A decision by the European Commission is expected within the next 2 months.
A version of this article first appeared on Medscape.com.
Which solid organ transplant recipients face the highest risk of skin cancer?
BOSTON – .
White patients who meet these criteria should be screening within 2 years after transplant, while Black patients should be screened within 5 years after transplant, Ally-Khan Somani, MD, PhD, said at the annual meeting of the American Academy of Dermatology.
Dr. Somani, director of dermatologic surgery and the division of cutaneous oncology at Indiana University, Indianapolis, based his remarks on consensus screening guidelines assembled from three rounds of Delphi method surveys with 47 dermatologists and 37 transplant physicians, with the goal of establishing skin cancer screening recommendations for SOTRs. Among the dermatologists surveyed, 45% were Mohs surgeons and 55% were general dermatologists.
The panel recommended that the transplant team should perform risk assessment for SOTRs to risk stratify patients for skin cancer screening (high risk vs. low risk). They also proposed that dermatologists perform skin cancer screening by full-body skin examinations, and that SOTRs with a history of skin cancer should continue with routine skin cancer surveillance as recommended by their dermatologists.
Those at low risk for skin cancer include abdominal organ recipients, SOTR age of younger than 50 at time of transplant, and female gender. The guidelines recommend that White, Asian, and Hispanic patients who meet those criteria should be screened within 5 years after transplant, while no consensus was reached for Black patients who meet those criteria.
Based on posttransplant skin cancer incidence rates, risk is increased among males, Whites, thoracic organ recipients, and being age 50 or older, Dr. Somani said. “At our institution, we make sure there’s a good connection between our transplant teams and dermatologists. We recommend rapid referral for suspicious lesions and we educate patients and screen them within 1 year of transplant, or sooner for high-risk patients. Surveillance is increased to every 3 or 4 months for patients with a history of multiple or high-risk cancers or sooner, followed by routine surveillance as recommended by the patient’s dermatologist.”
To risk stratify patients on the development of their first skin cancer post transplantation, researchers developed the Skin and Ultraviolet Neoplasia Transplant Risk Assessment Calculator (SUNTRAC), a prediction tool with a freely available app. Data for the tool were drawn from the Transplant Skin Cancer Network study, a 5-year analysis of 6,340 adult recipients of a first solid organ transplant at 26 transplant centers in the United States. It generates a risk score for SOTRs (low, medium, high, or very high), which informs transplant care providers of a patient’s risk of skin cancer.
Dr. Somani disclosed that he has received grants and funding from Castle Biosciences. He is an adviser to Cook Biotech and a consultant to Sanara MedTech.
BOSTON – .
White patients who meet these criteria should be screening within 2 years after transplant, while Black patients should be screened within 5 years after transplant, Ally-Khan Somani, MD, PhD, said at the annual meeting of the American Academy of Dermatology.
Dr. Somani, director of dermatologic surgery and the division of cutaneous oncology at Indiana University, Indianapolis, based his remarks on consensus screening guidelines assembled from three rounds of Delphi method surveys with 47 dermatologists and 37 transplant physicians, with the goal of establishing skin cancer screening recommendations for SOTRs. Among the dermatologists surveyed, 45% were Mohs surgeons and 55% were general dermatologists.
The panel recommended that the transplant team should perform risk assessment for SOTRs to risk stratify patients for skin cancer screening (high risk vs. low risk). They also proposed that dermatologists perform skin cancer screening by full-body skin examinations, and that SOTRs with a history of skin cancer should continue with routine skin cancer surveillance as recommended by their dermatologists.
Those at low risk for skin cancer include abdominal organ recipients, SOTR age of younger than 50 at time of transplant, and female gender. The guidelines recommend that White, Asian, and Hispanic patients who meet those criteria should be screened within 5 years after transplant, while no consensus was reached for Black patients who meet those criteria.
Based on posttransplant skin cancer incidence rates, risk is increased among males, Whites, thoracic organ recipients, and being age 50 or older, Dr. Somani said. “At our institution, we make sure there’s a good connection between our transplant teams and dermatologists. We recommend rapid referral for suspicious lesions and we educate patients and screen them within 1 year of transplant, or sooner for high-risk patients. Surveillance is increased to every 3 or 4 months for patients with a history of multiple or high-risk cancers or sooner, followed by routine surveillance as recommended by the patient’s dermatologist.”
To risk stratify patients on the development of their first skin cancer post transplantation, researchers developed the Skin and Ultraviolet Neoplasia Transplant Risk Assessment Calculator (SUNTRAC), a prediction tool with a freely available app. Data for the tool were drawn from the Transplant Skin Cancer Network study, a 5-year analysis of 6,340 adult recipients of a first solid organ transplant at 26 transplant centers in the United States. It generates a risk score for SOTRs (low, medium, high, or very high), which informs transplant care providers of a patient’s risk of skin cancer.
Dr. Somani disclosed that he has received grants and funding from Castle Biosciences. He is an adviser to Cook Biotech and a consultant to Sanara MedTech.
BOSTON – .
White patients who meet these criteria should be screening within 2 years after transplant, while Black patients should be screened within 5 years after transplant, Ally-Khan Somani, MD, PhD, said at the annual meeting of the American Academy of Dermatology.
Dr. Somani, director of dermatologic surgery and the division of cutaneous oncology at Indiana University, Indianapolis, based his remarks on consensus screening guidelines assembled from three rounds of Delphi method surveys with 47 dermatologists and 37 transplant physicians, with the goal of establishing skin cancer screening recommendations for SOTRs. Among the dermatologists surveyed, 45% were Mohs surgeons and 55% were general dermatologists.
The panel recommended that the transplant team should perform risk assessment for SOTRs to risk stratify patients for skin cancer screening (high risk vs. low risk). They also proposed that dermatologists perform skin cancer screening by full-body skin examinations, and that SOTRs with a history of skin cancer should continue with routine skin cancer surveillance as recommended by their dermatologists.
Those at low risk for skin cancer include abdominal organ recipients, SOTR age of younger than 50 at time of transplant, and female gender. The guidelines recommend that White, Asian, and Hispanic patients who meet those criteria should be screened within 5 years after transplant, while no consensus was reached for Black patients who meet those criteria.
Based on posttransplant skin cancer incidence rates, risk is increased among males, Whites, thoracic organ recipients, and being age 50 or older, Dr. Somani said. “At our institution, we make sure there’s a good connection between our transplant teams and dermatologists. We recommend rapid referral for suspicious lesions and we educate patients and screen them within 1 year of transplant, or sooner for high-risk patients. Surveillance is increased to every 3 or 4 months for patients with a history of multiple or high-risk cancers or sooner, followed by routine surveillance as recommended by the patient’s dermatologist.”
To risk stratify patients on the development of their first skin cancer post transplantation, researchers developed the Skin and Ultraviolet Neoplasia Transplant Risk Assessment Calculator (SUNTRAC), a prediction tool with a freely available app. Data for the tool were drawn from the Transplant Skin Cancer Network study, a 5-year analysis of 6,340 adult recipients of a first solid organ transplant at 26 transplant centers in the United States. It generates a risk score for SOTRs (low, medium, high, or very high), which informs transplant care providers of a patient’s risk of skin cancer.
Dr. Somani disclosed that he has received grants and funding from Castle Biosciences. He is an adviser to Cook Biotech and a consultant to Sanara MedTech.
AT AAD 22
Almost 60% of U.S. population has been infected by COVID-19: CDC
The percentage of Americans who have been infected with COVID-19 jumped from 34% in December 2021 to 58% in February 2022, a new study from the Centers for Disease Control and Prevention reveals.
This is the first time the seroprevalence of prior infection is more than 50% in the American population.
“I definitely expected that we were going to see an increase continue ... but I didn’t expect it to increase quite this much. But we follow the data ... and this is what the evidence is showing us,” lead study researcher Kristie E. N. Clarke, MD, said during a CDC media briefing April 26.
Researchers found that presence of antinucleocapsid (anti-N) antibodies from prior infection varied by age. The rate varied from as high as 75% in children and teenagers 17 years and younger to 33% in those 65 and older, for example.
The study showed that the anti-N antibodies were more common in age groups with the lowest vaccination numbers.
Combined with up-to-date CDC data on deaths, hospitalizations, and cases, the study provides a clearer picture of where we are now and where we might be headed in terms of the pandemic.
Vaccination still valuable
The fact that nearly 60% of Americans have antibodies from prior infection is not a reason to think people with a history of COVID-19 should skip vaccination, said CDC director Rochelle P. Walensky, MD.
“I can’t underscore enough that those with detectable antibodies from previous infection, we encourage them to still get vaccinated,” Dr. Walensky said.
“We do know that reinfections happen,” she said, “so that’s important in terms of thinking forward.”
The CDC continues to encourage all Americans to stay up to date with their COVID-19 vaccinations, said Dr. Clarke, colead for the CDC’s COVID-19 Epidemiology and Surveillance Taskforce Seroprevalence Team. “Having infection-induced antibodies does not necessarily mean you are protected against future infections.”
The study, published in the CDC’s Morbidity and Mortality Weekly Report (MMWR), did not evaluate antibody protection from COVID-19 vaccination.
It should also be noted that the study looked at presence or absence of anti-N antibodies, and not whether certain levels were linked to less or more protection.
Where are we now?
Dr. Walensky used the media briefing as an opportunity to share current COVID-19 numbers.
“Overall, we can continue to have some mixed trends. Deaths, fortunately, are continuing to trend downward with a 7-day average of about 300 per day, which represents an estimated 18% decline from the prior week,” she said.
Hospital admissions also remain low, at about 1,500 per day. “But we should note that for the second week in a row, they are slowly trending upwards,” Dr. Walensky said. There was an increase of about 9% at press time compared with the prior week.
Cases remain “comparatively low” to even where we were a month ago, at 44,000 per day,” Dr. Walensky said. “Although this too represents an increase of about 25% in the past week.”
Dr. Walensky noted that positive test numbers are not as reliable a metric as they were before the growth in use of rapid home tests. But it’s not the only measure. “We continue to believe that our PCR testing data, especially when we corroborate it with information from our other surveillance systems – like wastewater surveillance and emergency department surveillance – provide us a reliable picture of the trajectory of COVID-19 across our country.”
She recommended that people continue to consult the CDC’s COVID-19 county tracker to monitor local levels of COVID-19.
Dr. Walensky also shared recent findings from genomic sequencing that continue to show the predominance of the Omicron variant. “Essentially a hundred percent of what we’re finding now is Omicron,” she said. In terms of individual variants, the Omicron BA.1 variant is about 3% of circulating virus, the BA.2 variant is about 68%, and BA.2.12.1 makes up about 35%.
“We’re just starting to learn about the impact of BA2.121,” Dr. Walensky said. “It appears it might have a transmission advantage of about 25% over the BA2 subvariant.”
A version of this article first appeared on Medscape.com.
The percentage of Americans who have been infected with COVID-19 jumped from 34% in December 2021 to 58% in February 2022, a new study from the Centers for Disease Control and Prevention reveals.
This is the first time the seroprevalence of prior infection is more than 50% in the American population.
“I definitely expected that we were going to see an increase continue ... but I didn’t expect it to increase quite this much. But we follow the data ... and this is what the evidence is showing us,” lead study researcher Kristie E. N. Clarke, MD, said during a CDC media briefing April 26.
Researchers found that presence of antinucleocapsid (anti-N) antibodies from prior infection varied by age. The rate varied from as high as 75% in children and teenagers 17 years and younger to 33% in those 65 and older, for example.
The study showed that the anti-N antibodies were more common in age groups with the lowest vaccination numbers.
Combined with up-to-date CDC data on deaths, hospitalizations, and cases, the study provides a clearer picture of where we are now and where we might be headed in terms of the pandemic.
Vaccination still valuable
The fact that nearly 60% of Americans have antibodies from prior infection is not a reason to think people with a history of COVID-19 should skip vaccination, said CDC director Rochelle P. Walensky, MD.
“I can’t underscore enough that those with detectable antibodies from previous infection, we encourage them to still get vaccinated,” Dr. Walensky said.
“We do know that reinfections happen,” she said, “so that’s important in terms of thinking forward.”
The CDC continues to encourage all Americans to stay up to date with their COVID-19 vaccinations, said Dr. Clarke, colead for the CDC’s COVID-19 Epidemiology and Surveillance Taskforce Seroprevalence Team. “Having infection-induced antibodies does not necessarily mean you are protected against future infections.”
The study, published in the CDC’s Morbidity and Mortality Weekly Report (MMWR), did not evaluate antibody protection from COVID-19 vaccination.
It should also be noted that the study looked at presence or absence of anti-N antibodies, and not whether certain levels were linked to less or more protection.
Where are we now?
Dr. Walensky used the media briefing as an opportunity to share current COVID-19 numbers.
“Overall, we can continue to have some mixed trends. Deaths, fortunately, are continuing to trend downward with a 7-day average of about 300 per day, which represents an estimated 18% decline from the prior week,” she said.
Hospital admissions also remain low, at about 1,500 per day. “But we should note that for the second week in a row, they are slowly trending upwards,” Dr. Walensky said. There was an increase of about 9% at press time compared with the prior week.
Cases remain “comparatively low” to even where we were a month ago, at 44,000 per day,” Dr. Walensky said. “Although this too represents an increase of about 25% in the past week.”
Dr. Walensky noted that positive test numbers are not as reliable a metric as they were before the growth in use of rapid home tests. But it’s not the only measure. “We continue to believe that our PCR testing data, especially when we corroborate it with information from our other surveillance systems – like wastewater surveillance and emergency department surveillance – provide us a reliable picture of the trajectory of COVID-19 across our country.”
She recommended that people continue to consult the CDC’s COVID-19 county tracker to monitor local levels of COVID-19.
Dr. Walensky also shared recent findings from genomic sequencing that continue to show the predominance of the Omicron variant. “Essentially a hundred percent of what we’re finding now is Omicron,” she said. In terms of individual variants, the Omicron BA.1 variant is about 3% of circulating virus, the BA.2 variant is about 68%, and BA.2.12.1 makes up about 35%.
“We’re just starting to learn about the impact of BA2.121,” Dr. Walensky said. “It appears it might have a transmission advantage of about 25% over the BA2 subvariant.”
A version of this article first appeared on Medscape.com.
The percentage of Americans who have been infected with COVID-19 jumped from 34% in December 2021 to 58% in February 2022, a new study from the Centers for Disease Control and Prevention reveals.
This is the first time the seroprevalence of prior infection is more than 50% in the American population.
“I definitely expected that we were going to see an increase continue ... but I didn’t expect it to increase quite this much. But we follow the data ... and this is what the evidence is showing us,” lead study researcher Kristie E. N. Clarke, MD, said during a CDC media briefing April 26.
Researchers found that presence of antinucleocapsid (anti-N) antibodies from prior infection varied by age. The rate varied from as high as 75% in children and teenagers 17 years and younger to 33% in those 65 and older, for example.
The study showed that the anti-N antibodies were more common in age groups with the lowest vaccination numbers.
Combined with up-to-date CDC data on deaths, hospitalizations, and cases, the study provides a clearer picture of where we are now and where we might be headed in terms of the pandemic.
Vaccination still valuable
The fact that nearly 60% of Americans have antibodies from prior infection is not a reason to think people with a history of COVID-19 should skip vaccination, said CDC director Rochelle P. Walensky, MD.
“I can’t underscore enough that those with detectable antibodies from previous infection, we encourage them to still get vaccinated,” Dr. Walensky said.
“We do know that reinfections happen,” she said, “so that’s important in terms of thinking forward.”
The CDC continues to encourage all Americans to stay up to date with their COVID-19 vaccinations, said Dr. Clarke, colead for the CDC’s COVID-19 Epidemiology and Surveillance Taskforce Seroprevalence Team. “Having infection-induced antibodies does not necessarily mean you are protected against future infections.”
The study, published in the CDC’s Morbidity and Mortality Weekly Report (MMWR), did not evaluate antibody protection from COVID-19 vaccination.
It should also be noted that the study looked at presence or absence of anti-N antibodies, and not whether certain levels were linked to less or more protection.
Where are we now?
Dr. Walensky used the media briefing as an opportunity to share current COVID-19 numbers.
“Overall, we can continue to have some mixed trends. Deaths, fortunately, are continuing to trend downward with a 7-day average of about 300 per day, which represents an estimated 18% decline from the prior week,” she said.
Hospital admissions also remain low, at about 1,500 per day. “But we should note that for the second week in a row, they are slowly trending upwards,” Dr. Walensky said. There was an increase of about 9% at press time compared with the prior week.
Cases remain “comparatively low” to even where we were a month ago, at 44,000 per day,” Dr. Walensky said. “Although this too represents an increase of about 25% in the past week.”
Dr. Walensky noted that positive test numbers are not as reliable a metric as they were before the growth in use of rapid home tests. But it’s not the only measure. “We continue to believe that our PCR testing data, especially when we corroborate it with information from our other surveillance systems – like wastewater surveillance and emergency department surveillance – provide us a reliable picture of the trajectory of COVID-19 across our country.”
She recommended that people continue to consult the CDC’s COVID-19 county tracker to monitor local levels of COVID-19.
Dr. Walensky also shared recent findings from genomic sequencing that continue to show the predominance of the Omicron variant. “Essentially a hundred percent of what we’re finding now is Omicron,” she said. In terms of individual variants, the Omicron BA.1 variant is about 3% of circulating virus, the BA.2 variant is about 68%, and BA.2.12.1 makes up about 35%.
“We’re just starting to learn about the impact of BA2.121,” Dr. Walensky said. “It appears it might have a transmission advantage of about 25% over the BA2 subvariant.”
A version of this article first appeared on Medscape.com.
FROM MMWR
Erythematous Plaque on the Groin and Buttocks
The Diagnosis: Pseudomonas Pyoderma
A skin swab confirmed the presence of a ciprofloxacinsusceptible Pseudomonas aeruginosa strain. Our patient received oral ciprofloxacin 500 mg twice daily for 10 days with remarkable clinical improvement. The remaining skin lesion was successfully treated with more frequent diaper changes and the use of topical corticosteroids and emollients.
The topographical location, cutaneous morphology, clinical context, and sometimes the type of exudate are fundamental for the diagnosis of eruptions in intertriginous areas. Cutaneous Candida infections are common in these locations. They classically present as markedly erythematous plaques that occasionally are erosive, accompanied by satellite papules and pustules.1 Tinea cruris is a dermatophyte infection of the groin, proximal medial thighs, perineum, and buttocks. It usually presents as an erythematous patch that spreads centrifugally with partial central clearing and a slightly elevated, scaly border. Although candidiasis was higher on the differential, it was less likely, as our patient had a concomitant exudate inconsistent with Candida infections. Also, the lack of response to antifungal agents made hypotheses of fungal infections improbable.1
Inverse psoriasis is a variant of psoriasis identified by the development of well-demarcated, nonscaly, shiny plaques on body folds.2 Psoriasis is a chronic disease with several other cutaneous manifestations, such as nail and scalp involvement, as well as erythematous scaly plaques on the extensor surfaces of the limbs. The absence of a history of psoriasis, lack of other cutaneous manifestations, and no response to topical corticosteroids made the diagnosis of inverse psoriasis unlikely in our patient.
Erythrasma is a common superficial cutaneous infection caused by Corynebacterium minutissimum, a grampositive bacillus. It typically presents as an intertriginous eruption characterized by small erythematous to brown patches or thin plaques with fine scaling and sharp borders.3 Erythrasma displays a coral red fluorescence on Wood lamp examination that can be useful in the distinction from other causes of intertrigo.1 Although this examination had not been performed in our patient, the striking exudate made erythrasma less likely, and the culture performed on skin swab material would help to rule out this diagnosis.
Pseudomonas aeruginosa is a gram-negative strict aerobic bacillus of ubiquitous distribution with a preference for humid environments.4,5Pseudomonas aeruginosa infections were first reported in the 19th century by physicians who noticed a peculiar odorous condition that caused a blue-green discoloration on bandages. This coloration explains the species name aeruginosa which is derived from the Latin word for copper rust.4 It comes from several water-soluble pigments produced by this microorganism, the most prevalent of which are pyocyanin and pyoverdine. Pyocyanin has a greenish-blue color and is nonfluorescent, while pyoverdine is green-yellowish and fluoresces under Wood light.5 Other pigments, such as pyorubin and pyomelanin, can be produced by some Pseudomonas strains.4
Pseudomonas aeruginosa has become one of the main pathogens involved in hospital-acquired infections,6 especially in immunocompromised patients.6,7 It is a frequent cause of respiratory infections in patients with cystic fibrosis, as it is present in the airways of up to 70% of these patients in adulthood.7 Also, due to a variety of adaptive mechanisms with the development of resistance to a range of antibiotics, P aeruginosa has become a worldwide public health problem and is involved in several life-threatening nosocomial infections.7,8
Cutaneous P aeruginosa infections range from superficial to deep tissue involvement and can affect both immunocompromised and immunocompetent individuals.9 They are classified as primary when they originate directly from the skin or secondary when they occur in the context of bacteremia. Primary infections mostly are mild and often are seen in healthy individuals; they usually occur by inoculation and predominate in moist areas where skin breakdown is frequent. Secondary infections typically affect immunocompromised individuals and portend a poor prognosis.5,9
Denominated as Pseudomonas pyoderma, the superficial skin infection by P aeruginosa is described as a condition where the epidermis has a moth-eaten appearance with macerated or eroded borders.10 A blue-greenish exudate and a grape juice odor often are present. This infection usually occurs as a complication of several skin conditions such as tinea pedis, eczema, burns, wounds, and ulcers.5,10
We believe that our patient developed Pseudomonas pyoderma as a complication of diaper dermatitis. His extended hospital stay with the use of different antibiotic regimens for the treatment of several infectious complications may have contributed to the development of infection by P aeruginosa.11 Despite its great clinical relevance, there are few studies in the literature on primary skin infections caused by P aeruginosa, and clinical descriptions with images are rare. Our patient had a nonspecific noneczematous dermatitis, and the projections on the periphery of the lesion resembled the moth-eaten appearance of the classic description of Pseudomonas pyoderma.5,10 The presence of a greenish exudate should promptly raise suspicion for this entity. We believe that the presentation of this case can illustrate this finding and help physicians to recognize this infection.
- Kalra MG, Higgins KE, Kinney BS. Intertrigo and secondary skin infections. Am Fam Physician. 2014;89:569-573.
- Micali G, Verzi AE, Giuffrida G, et al. Inverse psoriasis: from diagnosis to current treatment options. Clin Cosmet Investig Dermatol. 2019; 12:953-959.
- Somerville DA. Erythrasma in normal young adults. J Med Microbiol. 1970;3:57-64.
- D’Agata E. Pseudomonas aeruginosa and other Pseudomonas species. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases. Vol 2. 8th ed. Elsevier; 2015:2518-2531.
- Silvestre JF, Betlloch MI. Cutaneous manifestations due to Pseudomonas infection. Int J Dermatol. 1999;38:419-431.
- Young LS, Armstrong D. Pseudomonas aeruginosa infections. CRC Crit Rev Clin Lab Sci. 1972;3:291-347.
- Moradali MF, Ghods S, Rehm BH. Pseudomonas aeruginosa lifestyle: a paradigm for adaptation, survival, and persistence. Front Cell Infect Microbiol. 2017;7:39.
- Rosenthal VD, Bat-Erdene I, Gupta D, et al. International Nosocomial Infection Control Consortium (INICC) report, data summary of 45 countries for 2012-2017: device-associated module. Am J Infect Control. 2020;48:423-432.
- Wu DC, Chan WW, Metelitsa AI, et al. Pseudomonas skin infection: clinical features, epidemiology, and management. Am J Clin Dermatol. 2011;12:157-169.
- Hall JH, Callaway JL, Tindall JP, et al. Pseudomonas aeruginosa in dermatology. Arch Dermatol. 1968;97:312-324.
- Merchant S, Proudfoot EM, Quadri HN, et al. Risk factors for Pseudomonas aeruginosa infections in Asia-Pacific and consequences of inappropriate initial antimicrobial therapy: a systematic literature review and meta-analysis. J Glob Antimicrob Resist. 2018;14:33-44.
The Diagnosis: Pseudomonas Pyoderma
A skin swab confirmed the presence of a ciprofloxacinsusceptible Pseudomonas aeruginosa strain. Our patient received oral ciprofloxacin 500 mg twice daily for 10 days with remarkable clinical improvement. The remaining skin lesion was successfully treated with more frequent diaper changes and the use of topical corticosteroids and emollients.
The topographical location, cutaneous morphology, clinical context, and sometimes the type of exudate are fundamental for the diagnosis of eruptions in intertriginous areas. Cutaneous Candida infections are common in these locations. They classically present as markedly erythematous plaques that occasionally are erosive, accompanied by satellite papules and pustules.1 Tinea cruris is a dermatophyte infection of the groin, proximal medial thighs, perineum, and buttocks. It usually presents as an erythematous patch that spreads centrifugally with partial central clearing and a slightly elevated, scaly border. Although candidiasis was higher on the differential, it was less likely, as our patient had a concomitant exudate inconsistent with Candida infections. Also, the lack of response to antifungal agents made hypotheses of fungal infections improbable.1
Inverse psoriasis is a variant of psoriasis identified by the development of well-demarcated, nonscaly, shiny plaques on body folds.2 Psoriasis is a chronic disease with several other cutaneous manifestations, such as nail and scalp involvement, as well as erythematous scaly plaques on the extensor surfaces of the limbs. The absence of a history of psoriasis, lack of other cutaneous manifestations, and no response to topical corticosteroids made the diagnosis of inverse psoriasis unlikely in our patient.
Erythrasma is a common superficial cutaneous infection caused by Corynebacterium minutissimum, a grampositive bacillus. It typically presents as an intertriginous eruption characterized by small erythematous to brown patches or thin plaques with fine scaling and sharp borders.3 Erythrasma displays a coral red fluorescence on Wood lamp examination that can be useful in the distinction from other causes of intertrigo.1 Although this examination had not been performed in our patient, the striking exudate made erythrasma less likely, and the culture performed on skin swab material would help to rule out this diagnosis.
Pseudomonas aeruginosa is a gram-negative strict aerobic bacillus of ubiquitous distribution with a preference for humid environments.4,5Pseudomonas aeruginosa infections were first reported in the 19th century by physicians who noticed a peculiar odorous condition that caused a blue-green discoloration on bandages. This coloration explains the species name aeruginosa which is derived from the Latin word for copper rust.4 It comes from several water-soluble pigments produced by this microorganism, the most prevalent of which are pyocyanin and pyoverdine. Pyocyanin has a greenish-blue color and is nonfluorescent, while pyoverdine is green-yellowish and fluoresces under Wood light.5 Other pigments, such as pyorubin and pyomelanin, can be produced by some Pseudomonas strains.4
Pseudomonas aeruginosa has become one of the main pathogens involved in hospital-acquired infections,6 especially in immunocompromised patients.6,7 It is a frequent cause of respiratory infections in patients with cystic fibrosis, as it is present in the airways of up to 70% of these patients in adulthood.7 Also, due to a variety of adaptive mechanisms with the development of resistance to a range of antibiotics, P aeruginosa has become a worldwide public health problem and is involved in several life-threatening nosocomial infections.7,8
Cutaneous P aeruginosa infections range from superficial to deep tissue involvement and can affect both immunocompromised and immunocompetent individuals.9 They are classified as primary when they originate directly from the skin or secondary when they occur in the context of bacteremia. Primary infections mostly are mild and often are seen in healthy individuals; they usually occur by inoculation and predominate in moist areas where skin breakdown is frequent. Secondary infections typically affect immunocompromised individuals and portend a poor prognosis.5,9
Denominated as Pseudomonas pyoderma, the superficial skin infection by P aeruginosa is described as a condition where the epidermis has a moth-eaten appearance with macerated or eroded borders.10 A blue-greenish exudate and a grape juice odor often are present. This infection usually occurs as a complication of several skin conditions such as tinea pedis, eczema, burns, wounds, and ulcers.5,10
We believe that our patient developed Pseudomonas pyoderma as a complication of diaper dermatitis. His extended hospital stay with the use of different antibiotic regimens for the treatment of several infectious complications may have contributed to the development of infection by P aeruginosa.11 Despite its great clinical relevance, there are few studies in the literature on primary skin infections caused by P aeruginosa, and clinical descriptions with images are rare. Our patient had a nonspecific noneczematous dermatitis, and the projections on the periphery of the lesion resembled the moth-eaten appearance of the classic description of Pseudomonas pyoderma.5,10 The presence of a greenish exudate should promptly raise suspicion for this entity. We believe that the presentation of this case can illustrate this finding and help physicians to recognize this infection.
The Diagnosis: Pseudomonas Pyoderma
A skin swab confirmed the presence of a ciprofloxacinsusceptible Pseudomonas aeruginosa strain. Our patient received oral ciprofloxacin 500 mg twice daily for 10 days with remarkable clinical improvement. The remaining skin lesion was successfully treated with more frequent diaper changes and the use of topical corticosteroids and emollients.
The topographical location, cutaneous morphology, clinical context, and sometimes the type of exudate are fundamental for the diagnosis of eruptions in intertriginous areas. Cutaneous Candida infections are common in these locations. They classically present as markedly erythematous plaques that occasionally are erosive, accompanied by satellite papules and pustules.1 Tinea cruris is a dermatophyte infection of the groin, proximal medial thighs, perineum, and buttocks. It usually presents as an erythematous patch that spreads centrifugally with partial central clearing and a slightly elevated, scaly border. Although candidiasis was higher on the differential, it was less likely, as our patient had a concomitant exudate inconsistent with Candida infections. Also, the lack of response to antifungal agents made hypotheses of fungal infections improbable.1
Inverse psoriasis is a variant of psoriasis identified by the development of well-demarcated, nonscaly, shiny plaques on body folds.2 Psoriasis is a chronic disease with several other cutaneous manifestations, such as nail and scalp involvement, as well as erythematous scaly plaques on the extensor surfaces of the limbs. The absence of a history of psoriasis, lack of other cutaneous manifestations, and no response to topical corticosteroids made the diagnosis of inverse psoriasis unlikely in our patient.
Erythrasma is a common superficial cutaneous infection caused by Corynebacterium minutissimum, a grampositive bacillus. It typically presents as an intertriginous eruption characterized by small erythematous to brown patches or thin plaques with fine scaling and sharp borders.3 Erythrasma displays a coral red fluorescence on Wood lamp examination that can be useful in the distinction from other causes of intertrigo.1 Although this examination had not been performed in our patient, the striking exudate made erythrasma less likely, and the culture performed on skin swab material would help to rule out this diagnosis.
Pseudomonas aeruginosa is a gram-negative strict aerobic bacillus of ubiquitous distribution with a preference for humid environments.4,5Pseudomonas aeruginosa infections were first reported in the 19th century by physicians who noticed a peculiar odorous condition that caused a blue-green discoloration on bandages. This coloration explains the species name aeruginosa which is derived from the Latin word for copper rust.4 It comes from several water-soluble pigments produced by this microorganism, the most prevalent of which are pyocyanin and pyoverdine. Pyocyanin has a greenish-blue color and is nonfluorescent, while pyoverdine is green-yellowish and fluoresces under Wood light.5 Other pigments, such as pyorubin and pyomelanin, can be produced by some Pseudomonas strains.4
Pseudomonas aeruginosa has become one of the main pathogens involved in hospital-acquired infections,6 especially in immunocompromised patients.6,7 It is a frequent cause of respiratory infections in patients with cystic fibrosis, as it is present in the airways of up to 70% of these patients in adulthood.7 Also, due to a variety of adaptive mechanisms with the development of resistance to a range of antibiotics, P aeruginosa has become a worldwide public health problem and is involved in several life-threatening nosocomial infections.7,8
Cutaneous P aeruginosa infections range from superficial to deep tissue involvement and can affect both immunocompromised and immunocompetent individuals.9 They are classified as primary when they originate directly from the skin or secondary when they occur in the context of bacteremia. Primary infections mostly are mild and often are seen in healthy individuals; they usually occur by inoculation and predominate in moist areas where skin breakdown is frequent. Secondary infections typically affect immunocompromised individuals and portend a poor prognosis.5,9
Denominated as Pseudomonas pyoderma, the superficial skin infection by P aeruginosa is described as a condition where the epidermis has a moth-eaten appearance with macerated or eroded borders.10 A blue-greenish exudate and a grape juice odor often are present. This infection usually occurs as a complication of several skin conditions such as tinea pedis, eczema, burns, wounds, and ulcers.5,10
We believe that our patient developed Pseudomonas pyoderma as a complication of diaper dermatitis. His extended hospital stay with the use of different antibiotic regimens for the treatment of several infectious complications may have contributed to the development of infection by P aeruginosa.11 Despite its great clinical relevance, there are few studies in the literature on primary skin infections caused by P aeruginosa, and clinical descriptions with images are rare. Our patient had a nonspecific noneczematous dermatitis, and the projections on the periphery of the lesion resembled the moth-eaten appearance of the classic description of Pseudomonas pyoderma.5,10 The presence of a greenish exudate should promptly raise suspicion for this entity. We believe that the presentation of this case can illustrate this finding and help physicians to recognize this infection.
- Kalra MG, Higgins KE, Kinney BS. Intertrigo and secondary skin infections. Am Fam Physician. 2014;89:569-573.
- Micali G, Verzi AE, Giuffrida G, et al. Inverse psoriasis: from diagnosis to current treatment options. Clin Cosmet Investig Dermatol. 2019; 12:953-959.
- Somerville DA. Erythrasma in normal young adults. J Med Microbiol. 1970;3:57-64.
- D’Agata E. Pseudomonas aeruginosa and other Pseudomonas species. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases. Vol 2. 8th ed. Elsevier; 2015:2518-2531.
- Silvestre JF, Betlloch MI. Cutaneous manifestations due to Pseudomonas infection. Int J Dermatol. 1999;38:419-431.
- Young LS, Armstrong D. Pseudomonas aeruginosa infections. CRC Crit Rev Clin Lab Sci. 1972;3:291-347.
- Moradali MF, Ghods S, Rehm BH. Pseudomonas aeruginosa lifestyle: a paradigm for adaptation, survival, and persistence. Front Cell Infect Microbiol. 2017;7:39.
- Rosenthal VD, Bat-Erdene I, Gupta D, et al. International Nosocomial Infection Control Consortium (INICC) report, data summary of 45 countries for 2012-2017: device-associated module. Am J Infect Control. 2020;48:423-432.
- Wu DC, Chan WW, Metelitsa AI, et al. Pseudomonas skin infection: clinical features, epidemiology, and management. Am J Clin Dermatol. 2011;12:157-169.
- Hall JH, Callaway JL, Tindall JP, et al. Pseudomonas aeruginosa in dermatology. Arch Dermatol. 1968;97:312-324.
- Merchant S, Proudfoot EM, Quadri HN, et al. Risk factors for Pseudomonas aeruginosa infections in Asia-Pacific and consequences of inappropriate initial antimicrobial therapy: a systematic literature review and meta-analysis. J Glob Antimicrob Resist. 2018;14:33-44.
- Kalra MG, Higgins KE, Kinney BS. Intertrigo and secondary skin infections. Am Fam Physician. 2014;89:569-573.
- Micali G, Verzi AE, Giuffrida G, et al. Inverse psoriasis: from diagnosis to current treatment options. Clin Cosmet Investig Dermatol. 2019; 12:953-959.
- Somerville DA. Erythrasma in normal young adults. J Med Microbiol. 1970;3:57-64.
- D’Agata E. Pseudomonas aeruginosa and other Pseudomonas species. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases. Vol 2. 8th ed. Elsevier; 2015:2518-2531.
- Silvestre JF, Betlloch MI. Cutaneous manifestations due to Pseudomonas infection. Int J Dermatol. 1999;38:419-431.
- Young LS, Armstrong D. Pseudomonas aeruginosa infections. CRC Crit Rev Clin Lab Sci. 1972;3:291-347.
- Moradali MF, Ghods S, Rehm BH. Pseudomonas aeruginosa lifestyle: a paradigm for adaptation, survival, and persistence. Front Cell Infect Microbiol. 2017;7:39.
- Rosenthal VD, Bat-Erdene I, Gupta D, et al. International Nosocomial Infection Control Consortium (INICC) report, data summary of 45 countries for 2012-2017: device-associated module. Am J Infect Control. 2020;48:423-432.
- Wu DC, Chan WW, Metelitsa AI, et al. Pseudomonas skin infection: clinical features, epidemiology, and management. Am J Clin Dermatol. 2011;12:157-169.
- Hall JH, Callaway JL, Tindall JP, et al. Pseudomonas aeruginosa in dermatology. Arch Dermatol. 1968;97:312-324.
- Merchant S, Proudfoot EM, Quadri HN, et al. Risk factors for Pseudomonas aeruginosa infections in Asia-Pacific and consequences of inappropriate initial antimicrobial therapy: a systematic literature review and meta-analysis. J Glob Antimicrob Resist. 2018;14:33-44.
A 68-year-old man presented with an extensive erythematous plaque of 3 weeks’ duration that started in the groin and spread to the buttocks. It was associated with pruritus and a burning sensation. He was admitted to the palliative care unit 1 year prior for the management of terminal lung cancer. Despite the use of topical corticosteroids and antifungals, the lesions gradually worsened with dissemination to the back. Physical examination revealed an erythematous macerated plaque that extended from the buttocks and groin region to the scapular area (top). Its borders had an eroded appearance with projections compatible with radial spread (bottom). A greenish exudate soaked the diaper and sheets. No other cutaneous lesions were noted.
Harlequin Syndrome: Discovery of an Ancient Schwannoma
To the Editor:
A 52-year-old man who was otherwise healthy and a long-distance runner presented with the sudden onset of diminished sweating on the left side of the body of 6 weeks’ duration. While training for a marathon, he reported that he perspired only on the right side of the body during runs of 12 to 15 miles; he observed a lack of sweating on the left side of the face, left side of the trunk, left arm, and left leg. This absence of sweating was accompanied by intense flushing on the right side of the face and trunk.
The patient did not take any medications. He reported no history of trauma and exhibited no neurologic deficits. A chest radiograph was negative. Thyroid function testing and a comprehensive metabolic panel were normal. Contrast-enhanced computed tomography of the chest and abdomen revealed a 4.3-cm soft-tissue mass in the left superior mediastinum that was superior to the aortic arch, posterior to the left subclavian artery in proximity to the sympathetic chain, and lateral to the trachea. The patient was diagnosed with Harlequin syndrome (HS).
Open thoracotomy was performed to remove the lesion. Analysis of the mass showed cystic areas, areas of hemorrhage (Figure 1A), and alternating zones of compact Antoni A spindle cells admixed with areas of less orderly Antoni B spindle cells within a hypocellular stroma (Figure 1B). Individual cells were characterized by eosinophilic cytoplasm and tapered nuclei. The mass appeared to be completely encapsulated. No mitotic figures were seen on multiple slides. The cells stained diffusely positive for S-100 proteins. At 6-month follow-up, the patient reported that he did not notice any return of normal sweating on the left side. However, the right-sided flushing had resolved.
Harlequin syndrome (also called the Harlequin sign) is a rare disorder of the sympathetic nervous system and should not be confused with lethal harlequin-type ichthyosis, an autosomal-recessive congenital disorder in which the affected newborn’s skin is hard and thickened over most of the body.1 Harlequin syndrome usually is characterized by unilateral flushing and sweating that can affect the face, trunk, and extremities.2 Physical stimuli, such as exercising (as in our patient), high body temperature, and the consumption of spicy or pungent food, or an emotional response can unmask or exacerbate symptoms of HS. The syndrome also can present with cluster headache.3 Harlequin syndrome is more common in females (66% of cases).4 Originally, the side of the face marked by increased sweating and flushing was perceived to be the pathologic side; now it is recognized that the anhidrotic side is affected by the causative pathology. The side of the face characterized by flushing might gradually darken as it compensates for lack of thermal regulation on the other side.2,5
Usually, HS is an idiopathic condition associated with localized failure of upper thoracic sympathetic chain ganglia.5 A theory is that HS is part of a spectrum of autoimmune autonomic ganglionopathy.6 Typically, the syndrome is asymptomatic at rest, but testing can reveal an underlying sympathetic lesion.7 Structural lesions have been reported as a cause of the syndrome,6 similar to our patient.
Disrupted thermoregulatory vasodilation in HS is caused by an ipsilateral lesion of the sympathetic vasodilator neurons that innervate the face. Hemifacial anhidrosis also occurs because sudomotor neurons travel within the same pathways as vasodilator neurons.4
Our patient had a posterior mediastinal ancient schwannoma to the left of the subclavian artery, lateral to the trachea, with ipsilateral anhidrosis of the forehead, cheek, chin, and torso. In the medical literature, the forehead, cheek, and chin are described as being affected in HS when the lesion is located under the bifurcation of the carotid artery.3,5 Most of the sudomotor and vasomotor fibers that innervate the face leave the spinal cord through ventral roots T2-T34 (symptomatic areas are described in Figure 2), which correlates with the hypothesis that HS results from a deficit originating in the third thoracic nerve that is caused by a peripheral lesion affecting sympathetic outflow through the third thoracic root.2 The location of our patient’s lesion supports this claim.
Harlequin syndrome can present simultaneously with ipsilateral Horner, Adie, and Ross syndromes.8 There are varying clinical presentations of Horner syndrome. Some patients with HS show autonomic ocular signs, such as miosis and ptosis, exhibiting Horner syndrome as an additional feature.5 Adie syndrome is characterized by tonic pupils with hyporeflexia and is unilateral in most cases. Ross syndrome is similar to Adie syndrome—including tonic pupils with hyporeflexia—in addition to a finding of segmental anhidrosis; it is bilateral in most cases.4
In some cases, Horner syndrome and HS originate from unilateral pharmaceutical sympathetic denervation (ie, as a consequence of paravertebral spread of local anesthetic to ipsilateral stellate ganglion).9 Facial nonflushing areas in HS typically are identical with anhidrotic areas10; Horner syndrome often is ipsilateral to the affected sympathetic region.11
Our patient exhibited secondary HS from a tumor effect; however, an underlying tumor or infarct is absent in many cases. In primary (idiopathic) cases of HS, treatment is not recommended because the syndrome is benign.10,11
If symptoms of HS cause notable social embarrassment, contralateral sympathectomy can be considered.5,12 Repeated stellate ganglion block with a local anesthetic could be a less invasive treatment option.13 When considered on a case-by-case-basis, botulinum toxin type A has been effective as a treatment of compensatory hyperhidrosis on the unaffected side.14
In cases of secondary HS, surgical removal of the lesion may alleviate symptoms, though thoracotomy in our patient to remove the schwannoma did not alleviate anhidrosis. The Table lists treatment options for primary and secondary HS.4,5,11
- Harlequin ichthyosis. MedlinePlus. National Library of Medicine [Internet]. Updated January 7, 2022. Accessed April 5, 2022. https://ghr.nlm.nih.gov/condition/harlequin-ichthyosis
- Lance JW, Drummond PD, Gandevia SC, et al. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psych. 1988;51:635-642. doi:10.1136/jnnp.51.5.635
- Lehman K, Kumar N, Vu Q, et al. Harlequin syndrome in cluster headache. Headache. 2016;56:1053-1054. doi:10.1111/head.12852
- Willaert WIM, Scheltinga MRM, Steenhuisen SF, et al. Harlequin syndrome: two new cases and a management proposal. Acta Neurol Belg. 2009;109:214-220.
- Duddy ME, Baker MR. Images in clinical medicine. Harlequin’s darker side. N Engl J Med. 2007;357:E22. doi:10.1056/NEJMicm067851
- Karam C. Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy. Auton Neurosci. 2016;194:58-59. doi:10.1016/j.autneu.2015.12.004
- Wasner G, Maag R, Ludwig J, et al. Harlequin syndrome—one face of many etiologies. Nat Clin Pract Neurol. 2005;1:54-59. doi:10.1038/ncpneuro0040
- Guilloton L, Demarquay G, Quesnel L, et al. Dysautonomic syndrome of the face with Harlequin sign and syndrome: three new cases and a review of the literature. Rev Neurol (Paris). 2013;169:884-891. doi:10.1016/j.neurol.2013.01.628
- Burlacu CL, Buggy DJ. Coexisting Harlequin and Horner syndromes after high thoracic paravertebral anaesthesia. Br J Anaesth. 2005;95:822-824. doi:10.1093/bja/aei258
- Morrison DA, Bibby K, Woodruff G. The “Harlequin” sign and congenital Horner’s syndrome. J Neurol Neurosurg Psych. 1997;62:626-628. doi:10.1136/jnnp.62.6.626
- Bremner F, Smith S. Pupillographic findings in 39 consecutive cases of Harlequin syndrome. J Neuroophthalmol. 2008;28:171-177. doi:10.1097/WNO.0b013e318183c885
- Kaur S, Aggarwal P, Jindal N, et al. Harlequin syndrome: a mask of rare dysautonomic syndromes. Dermatol Online J. 2015;21:13030/qt3q39d7mz.
- Reddy H, Fatah S, Gulve A, et al. Novel management of Harlequin syndrome with stellate ganglion block. Br J Dermatol. 2013;169:954-956. doi:10.1111/bjd.12561
- Manhdoi:10.1016/j.parkreldis.2015.11.030 RKJV, Spitz M, Vasconcellos LF. Botulinum toxin for treatment of Harlequin syndrome. Parkinsonism Relat Disord. 2016;23:112-113.
To the Editor:
A 52-year-old man who was otherwise healthy and a long-distance runner presented with the sudden onset of diminished sweating on the left side of the body of 6 weeks’ duration. While training for a marathon, he reported that he perspired only on the right side of the body during runs of 12 to 15 miles; he observed a lack of sweating on the left side of the face, left side of the trunk, left arm, and left leg. This absence of sweating was accompanied by intense flushing on the right side of the face and trunk.
The patient did not take any medications. He reported no history of trauma and exhibited no neurologic deficits. A chest radiograph was negative. Thyroid function testing and a comprehensive metabolic panel were normal. Contrast-enhanced computed tomography of the chest and abdomen revealed a 4.3-cm soft-tissue mass in the left superior mediastinum that was superior to the aortic arch, posterior to the left subclavian artery in proximity to the sympathetic chain, and lateral to the trachea. The patient was diagnosed with Harlequin syndrome (HS).
Open thoracotomy was performed to remove the lesion. Analysis of the mass showed cystic areas, areas of hemorrhage (Figure 1A), and alternating zones of compact Antoni A spindle cells admixed with areas of less orderly Antoni B spindle cells within a hypocellular stroma (Figure 1B). Individual cells were characterized by eosinophilic cytoplasm and tapered nuclei. The mass appeared to be completely encapsulated. No mitotic figures were seen on multiple slides. The cells stained diffusely positive for S-100 proteins. At 6-month follow-up, the patient reported that he did not notice any return of normal sweating on the left side. However, the right-sided flushing had resolved.
Harlequin syndrome (also called the Harlequin sign) is a rare disorder of the sympathetic nervous system and should not be confused with lethal harlequin-type ichthyosis, an autosomal-recessive congenital disorder in which the affected newborn’s skin is hard and thickened over most of the body.1 Harlequin syndrome usually is characterized by unilateral flushing and sweating that can affect the face, trunk, and extremities.2 Physical stimuli, such as exercising (as in our patient), high body temperature, and the consumption of spicy or pungent food, or an emotional response can unmask or exacerbate symptoms of HS. The syndrome also can present with cluster headache.3 Harlequin syndrome is more common in females (66% of cases).4 Originally, the side of the face marked by increased sweating and flushing was perceived to be the pathologic side; now it is recognized that the anhidrotic side is affected by the causative pathology. The side of the face characterized by flushing might gradually darken as it compensates for lack of thermal regulation on the other side.2,5
Usually, HS is an idiopathic condition associated with localized failure of upper thoracic sympathetic chain ganglia.5 A theory is that HS is part of a spectrum of autoimmune autonomic ganglionopathy.6 Typically, the syndrome is asymptomatic at rest, but testing can reveal an underlying sympathetic lesion.7 Structural lesions have been reported as a cause of the syndrome,6 similar to our patient.
Disrupted thermoregulatory vasodilation in HS is caused by an ipsilateral lesion of the sympathetic vasodilator neurons that innervate the face. Hemifacial anhidrosis also occurs because sudomotor neurons travel within the same pathways as vasodilator neurons.4
Our patient had a posterior mediastinal ancient schwannoma to the left of the subclavian artery, lateral to the trachea, with ipsilateral anhidrosis of the forehead, cheek, chin, and torso. In the medical literature, the forehead, cheek, and chin are described as being affected in HS when the lesion is located under the bifurcation of the carotid artery.3,5 Most of the sudomotor and vasomotor fibers that innervate the face leave the spinal cord through ventral roots T2-T34 (symptomatic areas are described in Figure 2), which correlates with the hypothesis that HS results from a deficit originating in the third thoracic nerve that is caused by a peripheral lesion affecting sympathetic outflow through the third thoracic root.2 The location of our patient’s lesion supports this claim.
Harlequin syndrome can present simultaneously with ipsilateral Horner, Adie, and Ross syndromes.8 There are varying clinical presentations of Horner syndrome. Some patients with HS show autonomic ocular signs, such as miosis and ptosis, exhibiting Horner syndrome as an additional feature.5 Adie syndrome is characterized by tonic pupils with hyporeflexia and is unilateral in most cases. Ross syndrome is similar to Adie syndrome—including tonic pupils with hyporeflexia—in addition to a finding of segmental anhidrosis; it is bilateral in most cases.4
In some cases, Horner syndrome and HS originate from unilateral pharmaceutical sympathetic denervation (ie, as a consequence of paravertebral spread of local anesthetic to ipsilateral stellate ganglion).9 Facial nonflushing areas in HS typically are identical with anhidrotic areas10; Horner syndrome often is ipsilateral to the affected sympathetic region.11
Our patient exhibited secondary HS from a tumor effect; however, an underlying tumor or infarct is absent in many cases. In primary (idiopathic) cases of HS, treatment is not recommended because the syndrome is benign.10,11
If symptoms of HS cause notable social embarrassment, contralateral sympathectomy can be considered.5,12 Repeated stellate ganglion block with a local anesthetic could be a less invasive treatment option.13 When considered on a case-by-case-basis, botulinum toxin type A has been effective as a treatment of compensatory hyperhidrosis on the unaffected side.14
In cases of secondary HS, surgical removal of the lesion may alleviate symptoms, though thoracotomy in our patient to remove the schwannoma did not alleviate anhidrosis. The Table lists treatment options for primary and secondary HS.4,5,11
To the Editor:
A 52-year-old man who was otherwise healthy and a long-distance runner presented with the sudden onset of diminished sweating on the left side of the body of 6 weeks’ duration. While training for a marathon, he reported that he perspired only on the right side of the body during runs of 12 to 15 miles; he observed a lack of sweating on the left side of the face, left side of the trunk, left arm, and left leg. This absence of sweating was accompanied by intense flushing on the right side of the face and trunk.
The patient did not take any medications. He reported no history of trauma and exhibited no neurologic deficits. A chest radiograph was negative. Thyroid function testing and a comprehensive metabolic panel were normal. Contrast-enhanced computed tomography of the chest and abdomen revealed a 4.3-cm soft-tissue mass in the left superior mediastinum that was superior to the aortic arch, posterior to the left subclavian artery in proximity to the sympathetic chain, and lateral to the trachea. The patient was diagnosed with Harlequin syndrome (HS).
Open thoracotomy was performed to remove the lesion. Analysis of the mass showed cystic areas, areas of hemorrhage (Figure 1A), and alternating zones of compact Antoni A spindle cells admixed with areas of less orderly Antoni B spindle cells within a hypocellular stroma (Figure 1B). Individual cells were characterized by eosinophilic cytoplasm and tapered nuclei. The mass appeared to be completely encapsulated. No mitotic figures were seen on multiple slides. The cells stained diffusely positive for S-100 proteins. At 6-month follow-up, the patient reported that he did not notice any return of normal sweating on the left side. However, the right-sided flushing had resolved.
Harlequin syndrome (also called the Harlequin sign) is a rare disorder of the sympathetic nervous system and should not be confused with lethal harlequin-type ichthyosis, an autosomal-recessive congenital disorder in which the affected newborn’s skin is hard and thickened over most of the body.1 Harlequin syndrome usually is characterized by unilateral flushing and sweating that can affect the face, trunk, and extremities.2 Physical stimuli, such as exercising (as in our patient), high body temperature, and the consumption of spicy or pungent food, or an emotional response can unmask or exacerbate symptoms of HS. The syndrome also can present with cluster headache.3 Harlequin syndrome is more common in females (66% of cases).4 Originally, the side of the face marked by increased sweating and flushing was perceived to be the pathologic side; now it is recognized that the anhidrotic side is affected by the causative pathology. The side of the face characterized by flushing might gradually darken as it compensates for lack of thermal regulation on the other side.2,5
Usually, HS is an idiopathic condition associated with localized failure of upper thoracic sympathetic chain ganglia.5 A theory is that HS is part of a spectrum of autoimmune autonomic ganglionopathy.6 Typically, the syndrome is asymptomatic at rest, but testing can reveal an underlying sympathetic lesion.7 Structural lesions have been reported as a cause of the syndrome,6 similar to our patient.
Disrupted thermoregulatory vasodilation in HS is caused by an ipsilateral lesion of the sympathetic vasodilator neurons that innervate the face. Hemifacial anhidrosis also occurs because sudomotor neurons travel within the same pathways as vasodilator neurons.4
Our patient had a posterior mediastinal ancient schwannoma to the left of the subclavian artery, lateral to the trachea, with ipsilateral anhidrosis of the forehead, cheek, chin, and torso. In the medical literature, the forehead, cheek, and chin are described as being affected in HS when the lesion is located under the bifurcation of the carotid artery.3,5 Most of the sudomotor and vasomotor fibers that innervate the face leave the spinal cord through ventral roots T2-T34 (symptomatic areas are described in Figure 2), which correlates with the hypothesis that HS results from a deficit originating in the third thoracic nerve that is caused by a peripheral lesion affecting sympathetic outflow through the third thoracic root.2 The location of our patient’s lesion supports this claim.
Harlequin syndrome can present simultaneously with ipsilateral Horner, Adie, and Ross syndromes.8 There are varying clinical presentations of Horner syndrome. Some patients with HS show autonomic ocular signs, such as miosis and ptosis, exhibiting Horner syndrome as an additional feature.5 Adie syndrome is characterized by tonic pupils with hyporeflexia and is unilateral in most cases. Ross syndrome is similar to Adie syndrome—including tonic pupils with hyporeflexia—in addition to a finding of segmental anhidrosis; it is bilateral in most cases.4
In some cases, Horner syndrome and HS originate from unilateral pharmaceutical sympathetic denervation (ie, as a consequence of paravertebral spread of local anesthetic to ipsilateral stellate ganglion).9 Facial nonflushing areas in HS typically are identical with anhidrotic areas10; Horner syndrome often is ipsilateral to the affected sympathetic region.11
Our patient exhibited secondary HS from a tumor effect; however, an underlying tumor or infarct is absent in many cases. In primary (idiopathic) cases of HS, treatment is not recommended because the syndrome is benign.10,11
If symptoms of HS cause notable social embarrassment, contralateral sympathectomy can be considered.5,12 Repeated stellate ganglion block with a local anesthetic could be a less invasive treatment option.13 When considered on a case-by-case-basis, botulinum toxin type A has been effective as a treatment of compensatory hyperhidrosis on the unaffected side.14
In cases of secondary HS, surgical removal of the lesion may alleviate symptoms, though thoracotomy in our patient to remove the schwannoma did not alleviate anhidrosis. The Table lists treatment options for primary and secondary HS.4,5,11
- Harlequin ichthyosis. MedlinePlus. National Library of Medicine [Internet]. Updated January 7, 2022. Accessed April 5, 2022. https://ghr.nlm.nih.gov/condition/harlequin-ichthyosis
- Lance JW, Drummond PD, Gandevia SC, et al. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psych. 1988;51:635-642. doi:10.1136/jnnp.51.5.635
- Lehman K, Kumar N, Vu Q, et al. Harlequin syndrome in cluster headache. Headache. 2016;56:1053-1054. doi:10.1111/head.12852
- Willaert WIM, Scheltinga MRM, Steenhuisen SF, et al. Harlequin syndrome: two new cases and a management proposal. Acta Neurol Belg. 2009;109:214-220.
- Duddy ME, Baker MR. Images in clinical medicine. Harlequin’s darker side. N Engl J Med. 2007;357:E22. doi:10.1056/NEJMicm067851
- Karam C. Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy. Auton Neurosci. 2016;194:58-59. doi:10.1016/j.autneu.2015.12.004
- Wasner G, Maag R, Ludwig J, et al. Harlequin syndrome—one face of many etiologies. Nat Clin Pract Neurol. 2005;1:54-59. doi:10.1038/ncpneuro0040
- Guilloton L, Demarquay G, Quesnel L, et al. Dysautonomic syndrome of the face with Harlequin sign and syndrome: three new cases and a review of the literature. Rev Neurol (Paris). 2013;169:884-891. doi:10.1016/j.neurol.2013.01.628
- Burlacu CL, Buggy DJ. Coexisting Harlequin and Horner syndromes after high thoracic paravertebral anaesthesia. Br J Anaesth. 2005;95:822-824. doi:10.1093/bja/aei258
- Morrison DA, Bibby K, Woodruff G. The “Harlequin” sign and congenital Horner’s syndrome. J Neurol Neurosurg Psych. 1997;62:626-628. doi:10.1136/jnnp.62.6.626
- Bremner F, Smith S. Pupillographic findings in 39 consecutive cases of Harlequin syndrome. J Neuroophthalmol. 2008;28:171-177. doi:10.1097/WNO.0b013e318183c885
- Kaur S, Aggarwal P, Jindal N, et al. Harlequin syndrome: a mask of rare dysautonomic syndromes. Dermatol Online J. 2015;21:13030/qt3q39d7mz.
- Reddy H, Fatah S, Gulve A, et al. Novel management of Harlequin syndrome with stellate ganglion block. Br J Dermatol. 2013;169:954-956. doi:10.1111/bjd.12561
- Manhdoi:10.1016/j.parkreldis.2015.11.030 RKJV, Spitz M, Vasconcellos LF. Botulinum toxin for treatment of Harlequin syndrome. Parkinsonism Relat Disord. 2016;23:112-113.
- Harlequin ichthyosis. MedlinePlus. National Library of Medicine [Internet]. Updated January 7, 2022. Accessed April 5, 2022. https://ghr.nlm.nih.gov/condition/harlequin-ichthyosis
- Lance JW, Drummond PD, Gandevia SC, et al. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psych. 1988;51:635-642. doi:10.1136/jnnp.51.5.635
- Lehman K, Kumar N, Vu Q, et al. Harlequin syndrome in cluster headache. Headache. 2016;56:1053-1054. doi:10.1111/head.12852
- Willaert WIM, Scheltinga MRM, Steenhuisen SF, et al. Harlequin syndrome: two new cases and a management proposal. Acta Neurol Belg. 2009;109:214-220.
- Duddy ME, Baker MR. Images in clinical medicine. Harlequin’s darker side. N Engl J Med. 2007;357:E22. doi:10.1056/NEJMicm067851
- Karam C. Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy. Auton Neurosci. 2016;194:58-59. doi:10.1016/j.autneu.2015.12.004
- Wasner G, Maag R, Ludwig J, et al. Harlequin syndrome—one face of many etiologies. Nat Clin Pract Neurol. 2005;1:54-59. doi:10.1038/ncpneuro0040
- Guilloton L, Demarquay G, Quesnel L, et al. Dysautonomic syndrome of the face with Harlequin sign and syndrome: three new cases and a review of the literature. Rev Neurol (Paris). 2013;169:884-891. doi:10.1016/j.neurol.2013.01.628
- Burlacu CL, Buggy DJ. Coexisting Harlequin and Horner syndromes after high thoracic paravertebral anaesthesia. Br J Anaesth. 2005;95:822-824. doi:10.1093/bja/aei258
- Morrison DA, Bibby K, Woodruff G. The “Harlequin” sign and congenital Horner’s syndrome. J Neurol Neurosurg Psych. 1997;62:626-628. doi:10.1136/jnnp.62.6.626
- Bremner F, Smith S. Pupillographic findings in 39 consecutive cases of Harlequin syndrome. J Neuroophthalmol. 2008;28:171-177. doi:10.1097/WNO.0b013e318183c885
- Kaur S, Aggarwal P, Jindal N, et al. Harlequin syndrome: a mask of rare dysautonomic syndromes. Dermatol Online J. 2015;21:13030/qt3q39d7mz.
- Reddy H, Fatah S, Gulve A, et al. Novel management of Harlequin syndrome with stellate ganglion block. Br J Dermatol. 2013;169:954-956. doi:10.1111/bjd.12561
- Manhdoi:10.1016/j.parkreldis.2015.11.030 RKJV, Spitz M, Vasconcellos LF. Botulinum toxin for treatment of Harlequin syndrome. Parkinsonism Relat Disord. 2016;23:112-113.
Practice Points
- Harlequin syndrome is a rare disorder of the sympathetic nervous system that is characterized by unilateral flushing and sweating that can affect the face, trunk, and extremities.
- Secondary causes can be from schwannomas in the cervical chain ganglion.