Dermatology

Children with juvenile dermatomyositis showed significant growth failure and pubertal delay, based on data from a longitudinal cohort study.

welcomia/iStock/Getty Images Plus

“Both the inflammatory activity of this severe chronic rheumatic disease and the well-known side effects of corticosteroid treatment may interfere with normal growth and pubertal development of children,” wrote Ellen Nordal, MD, of the University Hospital of Northern Norway, Tromsø, and colleagues.

The goal in treating juvenile dermatomyositis (JDM) is to achieve inactive disease and prevent permanent damage, but long-term data on growth and puberty in JDM patients are limited, they wrote.

In a study published in Arthritis Care & Research, the investigators reviewed data from 196 children and followed them for 2 years. The patients were part of the Paediatric Rheumatology International Trials Organisation (PRINTO) observational cohort study.

Overall, the researchers identified growth failure, height deflection, and/or delayed puberty in 94 children (48%) at the last study visit.

Growth failure was present at baseline in 17% of girls and 10% of boys. Over the 2-year study period, height deflection increased to 25% of girls and 31% of boys, but this change was not significant. Height deflection was defined as a change in the height z score of less than –0.25 per year from baseline. However, body mass index increased significantly from baseline during the study.

Catch-up growth had occurred by the final study visit in some patients, based on parent-adjusted z scores over time. Girls with a disease duration of 12 months or more showed no catch-up growth at 2 years and had significantly lower parent-adjusted height z scores.

In addition, the researchers observed a delay in the onset of puberty (including pubertal tempo and menarche) in approximately 36% of both boys and girls. However, neither growth failure nor height deflection was significantly associated with delayed puberty in either sex.

“In follow-up, clinicians should therefore be aware of both the pubertal development and the growth of the child, assess the milestones of development, and ensure that the children reach as much as possible of their genetic potential,” the researchers wrote.

The study participants were younger than 18 years at study enrollment, and all were in an active disease phase, defined as needing to start or receive a major dose increase of corticosteroids and/or immunosuppressants. Patients were assessed at baseline, at 6 months and/or at 12 months, and during a final visit at approximately 26 months. During the study, approximately half of the participants (50.5%) received methotrexate, 30 (15.3%) received cyclosporine A, 10 (5.1%) received cyclophosphamide, and 27 (13.8%) received intravenous immunoglobulin.

The study findings were limited by several factors, including the short follow-up period for assessing pubertal development and the inability to analyze any impact of corticosteroid use prior to the study, the researchers noted. However, “the overall frequency of growth failure was not significantly higher at the final study visit 2 years after baseline, indicating that the very high doses of corticosteroid treatment given during the study period is reasonably well tolerated with regards to growth,” they wrote. But monitoring remains essential, especially for children with previous growth failure or with disease onset early in pubertal development.

The study was supported by the European Union, Helse Nord Research grants, and by IRCCS Istituto Giannina Gaslini. Five authors of the study reported financial relationships with pharmaceutical companies.

SOURCE: Nordal E et al. Arthritis Care Res. 2019 Sep 10. doi: 10.1002/acr.24065.

Children with juvenile dermatomyositis showed significant growth failure and pubertal delay, based on data from a longitudinal cohort study.

welcomia/iStock/Getty Images Plus

“Both the inflammatory activity of this severe chronic rheumatic disease and the well-known side effects of corticosteroid treatment may interfere with normal growth and pubertal development of children,” wrote Ellen Nordal, MD, of the University Hospital of Northern Norway, Tromsø, and colleagues.

The goal in treating juvenile dermatomyositis (JDM) is to achieve inactive disease and prevent permanent damage, but long-term data on growth and puberty in JDM patients are limited, they wrote.

In a study published in Arthritis Care & Research, the investigators reviewed data from 196 children and followed them for 2 years. The patients were part of the Paediatric Rheumatology International Trials Organisation (PRINTO) observational cohort study.

Overall, the researchers identified growth failure, height deflection, and/or delayed puberty in 94 children (48%) at the last study visit.

Growth failure was present at baseline in 17% of girls and 10% of boys. Over the 2-year study period, height deflection increased to 25% of girls and 31% of boys, but this change was not significant. Height deflection was defined as a change in the height z score of less than –0.25 per year from baseline. However, body mass index increased significantly from baseline during the study.

Catch-up growth had occurred by the final study visit in some patients, based on parent-adjusted z scores over time. Girls with a disease duration of 12 months or more showed no catch-up growth at 2 years and had significantly lower parent-adjusted height z scores.

In addition, the researchers observed a delay in the onset of puberty (including pubertal tempo and menarche) in approximately 36% of both boys and girls. However, neither growth failure nor height deflection was significantly associated with delayed puberty in either sex.

“In follow-up, clinicians should therefore be aware of both the pubertal development and the growth of the child, assess the milestones of development, and ensure that the children reach as much as possible of their genetic potential,” the researchers wrote.

The study participants were younger than 18 years at study enrollment, and all were in an active disease phase, defined as needing to start or receive a major dose increase of corticosteroids and/or immunosuppressants. Patients were assessed at baseline, at 6 months and/or at 12 months, and during a final visit at approximately 26 months. During the study, approximately half of the participants (50.5%) received methotrexate, 30 (15.3%) received cyclosporine A, 10 (5.1%) received cyclophosphamide, and 27 (13.8%) received intravenous immunoglobulin.

The study findings were limited by several factors, including the short follow-up period for assessing pubertal development and the inability to analyze any impact of corticosteroid use prior to the study, the researchers noted. However, “the overall frequency of growth failure was not significantly higher at the final study visit 2 years after baseline, indicating that the very high doses of corticosteroid treatment given during the study period is reasonably well tolerated with regards to growth,” they wrote. But monitoring remains essential, especially for children with previous growth failure or with disease onset early in pubertal development.

The study was supported by the European Union, Helse Nord Research grants, and by IRCCS Istituto Giannina Gaslini. Five authors of the study reported financial relationships with pharmaceutical companies.

SOURCE: Nordal E et al. Arthritis Care Res. 2019 Sep 10. doi: 10.1002/acr.24065.

Children with juvenile dermatomyositis showed significant growth failure and pubertal delay, based on data from a longitudinal cohort study.

welcomia/iStock/Getty Images Plus

“Both the inflammatory activity of this severe chronic rheumatic disease and the well-known side effects of corticosteroid treatment may interfere with normal growth and pubertal development of children,” wrote Ellen Nordal, MD, of the University Hospital of Northern Norway, Tromsø, and colleagues.

The goal in treating juvenile dermatomyositis (JDM) is to achieve inactive disease and prevent permanent damage, but long-term data on growth and puberty in JDM patients are limited, they wrote.

In a study published in Arthritis Care & Research, the investigators reviewed data from 196 children and followed them for 2 years. The patients were part of the Paediatric Rheumatology International Trials Organisation (PRINTO) observational cohort study.

Overall, the researchers identified growth failure, height deflection, and/or delayed puberty in 94 children (48%) at the last study visit.

Growth failure was present at baseline in 17% of girls and 10% of boys. Over the 2-year study period, height deflection increased to 25% of girls and 31% of boys, but this change was not significant. Height deflection was defined as a change in the height z score of less than –0.25 per year from baseline. However, body mass index increased significantly from baseline during the study.

Catch-up growth had occurred by the final study visit in some patients, based on parent-adjusted z scores over time. Girls with a disease duration of 12 months or more showed no catch-up growth at 2 years and had significantly lower parent-adjusted height z scores.

In addition, the researchers observed a delay in the onset of puberty (including pubertal tempo and menarche) in approximately 36% of both boys and girls. However, neither growth failure nor height deflection was significantly associated with delayed puberty in either sex.

“In follow-up, clinicians should therefore be aware of both the pubertal development and the growth of the child, assess the milestones of development, and ensure that the children reach as much as possible of their genetic potential,” the researchers wrote.

The study participants were younger than 18 years at study enrollment, and all were in an active disease phase, defined as needing to start or receive a major dose increase of corticosteroids and/or immunosuppressants. Patients were assessed at baseline, at 6 months and/or at 12 months, and during a final visit at approximately 26 months. During the study, approximately half of the participants (50.5%) received methotrexate, 30 (15.3%) received cyclosporine A, 10 (5.1%) received cyclophosphamide, and 27 (13.8%) received intravenous immunoglobulin.

The study findings were limited by several factors, including the short follow-up period for assessing pubertal development and the inability to analyze any impact of corticosteroid use prior to the study, the researchers noted. However, “the overall frequency of growth failure was not significantly higher at the final study visit 2 years after baseline, indicating that the very high doses of corticosteroid treatment given during the study period is reasonably well tolerated with regards to growth,” they wrote. But monitoring remains essential, especially for children with previous growth failure or with disease onset early in pubertal development.

The study was supported by the European Union, Helse Nord Research grants, and by IRCCS Istituto Giannina Gaslini. Five authors of the study reported financial relationships with pharmaceutical companies.

SOURCE: Nordal E et al. Arthritis Care Res. 2019 Sep 10. doi: 10.1002/acr.24065.

Mycoplasma pneumoniae infection commonly manifests as an upper or lower respiratory tract infection with associated fever, dyspnea, cough, and coryza. However, patients can present with extrapulmonary complications with dermatologic findings including mucocutaneous eruptions. M. pneumoniae–associated mucocutaneous disease has prominent mucositis and typically sparse cutaneous involvement. The mucositis usually involves the lips and oral mucosa, eye conjunctivae, and nasal mucosa and can involve urogenital lesions. It predominantly is observed in children and adolescents. This condition is essentially a subtype of Stevens-Johnson syndrome, with a specific infection-associated etiology, and has been called “Mycoplasma pneumoniae–induced rash and mucositis,” shortened to “MIRM.”

Severe reactive mucocutaneous eruptions include erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). While there has been semantic confusion over the years, there are some distinctive characteristics.

EM is characterized by typical three-ringed target papules that are predominantly acral in location and often without mucosal involvement. The lesions are “multiforme” in that they can appear polymorphous and evolve during an episode, with erythematous macules progressing to edematous papules, sometimes with a halo of pallor and concentric “target-like” appearance. Lesions of EM are fixed, meaning individual lesions last 7-10 days, unlike urticarial lesions that last hours. EM classically is associated with herpes simplex virus infections which usually precede its development.

SJS and TEN display atypical macules and papules which develop into erythematous vesicles, bullae, and potentially extensive desquamation, usually presenting with fever and systemic symptoms, with multiple mucosal sites involved. SJS usually is defined by having bullae restricted to less than 10% of body surface area (BSA), TEN as greater than 30% BSA, and “overlap SJS-TEN” as 20%-30% skin detachment.¹ SJS and TEN commonly are induced by medications and on a spectrum of drug hypersensitivity–induced epidermal necrolysis.

MIRM has been highlighted as a distinct, common condition, usually mucous-membrane predominant with involvement of two or more mucosal sites, less than 10% total BSA, the presence of few vesiculobullous lesions or scattered atypical targets with or without targetoid lesions (without rash is called MIRM sine rash), and clinical and laboratory evidence of atypical pneumonia.² Other infections can cause similar eruptions (for example, Chlamydia pneumoniae), and a recent proposal by the Pediatric Dermatology Research Alliance has suggested the term “Reactive Infectious Mucocutaneous Eruption” (RIME) to include MIRM and other infection-induced reactions.

Laboratory diagnosis of M. pneumoniae is via serology or polymerase chain reaction. Antibody titers begin to rise approximately 7-9 days after infection and peak at 3-4 weeks. Enzyme immunoassay is more sensitive in detecting acute infection than culture and has sensitivity comparable to the polymerase chain reaction if there has been sufficient time to develop an antibody response.

Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Dr. Bhatti is a research fellow in pediatric dermatology at Rady Children’s Hospital and the University of California, San Diego. They said they have no financial disclosures. Email Dr. Eichenfield and Dr. Bhatti at [email protected].

References

1. Arch Dermatol. 1993 Jan;129(1):92-6.

2. J Am Acad Dermatol. 2015 Feb;72(2):239-45.

Mycoplasma pneumoniae infection commonly manifests as an upper or lower respiratory tract infection with associated fever, dyspnea, cough, and coryza. However, patients can present with extrapulmonary complications with dermatologic findings including mucocutaneous eruptions. M. pneumoniae–associated mucocutaneous disease has prominent mucositis and typically sparse cutaneous involvement. The mucositis usually involves the lips and oral mucosa, eye conjunctivae, and nasal mucosa and can involve urogenital lesions. It predominantly is observed in children and adolescents. This condition is essentially a subtype of Stevens-Johnson syndrome, with a specific infection-associated etiology, and has been called “Mycoplasma pneumoniae–induced rash and mucositis,” shortened to “MIRM.”

Severe reactive mucocutaneous eruptions include erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). While there has been semantic confusion over the years, there are some distinctive characteristics.

EM is characterized by typical three-ringed target papules that are predominantly acral in location and often without mucosal involvement. The lesions are “multiforme” in that they can appear polymorphous and evolve during an episode, with erythematous macules progressing to edematous papules, sometimes with a halo of pallor and concentric “target-like” appearance. Lesions of EM are fixed, meaning individual lesions last 7-10 days, unlike urticarial lesions that last hours. EM classically is associated with herpes simplex virus infections which usually precede its development.

SJS and TEN display atypical macules and papules which develop into erythematous vesicles, bullae, and potentially extensive desquamation, usually presenting with fever and systemic symptoms, with multiple mucosal sites involved. SJS usually is defined by having bullae restricted to less than 10% of body surface area (BSA), TEN as greater than 30% BSA, and “overlap SJS-TEN” as 20%-30% skin detachment.¹ SJS and TEN commonly are induced by medications and on a spectrum of drug hypersensitivity–induced epidermal necrolysis.

MIRM has been highlighted as a distinct, common condition, usually mucous-membrane predominant with involvement of two or more mucosal sites, less than 10% total BSA, the presence of few vesiculobullous lesions or scattered atypical targets with or without targetoid lesions (without rash is called MIRM sine rash), and clinical and laboratory evidence of atypical pneumonia.² Other infections can cause similar eruptions (for example, Chlamydia pneumoniae), and a recent proposal by the Pediatric Dermatology Research Alliance has suggested the term “Reactive Infectious Mucocutaneous Eruption” (RIME) to include MIRM and other infection-induced reactions.

Laboratory diagnosis of M. pneumoniae is via serology or polymerase chain reaction. Antibody titers begin to rise approximately 7-9 days after infection and peak at 3-4 weeks. Enzyme immunoassay is more sensitive in detecting acute infection than culture and has sensitivity comparable to the polymerase chain reaction if there has been sufficient time to develop an antibody response.

Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Dr. Bhatti is a research fellow in pediatric dermatology at Rady Children’s Hospital and the University of California, San Diego. They said they have no financial disclosures. Email Dr. Eichenfield and Dr. Bhatti at [email protected].

References

1. Arch Dermatol. 1993 Jan;129(1):92-6.

2. J Am Acad Dermatol. 2015 Feb;72(2):239-45.

Mycoplasma pneumoniae infection commonly manifests as an upper or lower respiratory tract infection with associated fever, dyspnea, cough, and coryza. However, patients can present with extrapulmonary complications with dermatologic findings including mucocutaneous eruptions. M. pneumoniae–associated mucocutaneous disease has prominent mucositis and typically sparse cutaneous involvement. The mucositis usually involves the lips and oral mucosa, eye conjunctivae, and nasal mucosa and can involve urogenital lesions. It predominantly is observed in children and adolescents. This condition is essentially a subtype of Stevens-Johnson syndrome, with a specific infection-associated etiology, and has been called “Mycoplasma pneumoniae–induced rash and mucositis,” shortened to “MIRM.”

Severe reactive mucocutaneous eruptions include erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). While there has been semantic confusion over the years, there are some distinctive characteristics.

EM is characterized by typical three-ringed target papules that are predominantly acral in location and often without mucosal involvement. The lesions are “multiforme” in that they can appear polymorphous and evolve during an episode, with erythematous macules progressing to edematous papules, sometimes with a halo of pallor and concentric “target-like” appearance. Lesions of EM are fixed, meaning individual lesions last 7-10 days, unlike urticarial lesions that last hours. EM classically is associated with herpes simplex virus infections which usually precede its development.

SJS and TEN display atypical macules and papules which develop into erythematous vesicles, bullae, and potentially extensive desquamation, usually presenting with fever and systemic symptoms, with multiple mucosal sites involved. SJS usually is defined by having bullae restricted to less than 10% of body surface area (BSA), TEN as greater than 30% BSA, and “overlap SJS-TEN” as 20%-30% skin detachment.¹ SJS and TEN commonly are induced by medications and on a spectrum of drug hypersensitivity–induced epidermal necrolysis.

MIRM has been highlighted as a distinct, common condition, usually mucous-membrane predominant with involvement of two or more mucosal sites, less than 10% total BSA, the presence of few vesiculobullous lesions or scattered atypical targets with or without targetoid lesions (without rash is called MIRM sine rash), and clinical and laboratory evidence of atypical pneumonia.² Other infections can cause similar eruptions (for example, Chlamydia pneumoniae), and a recent proposal by the Pediatric Dermatology Research Alliance has suggested the term “Reactive Infectious Mucocutaneous Eruption” (RIME) to include MIRM and other infection-induced reactions.

Laboratory diagnosis of M. pneumoniae is via serology or polymerase chain reaction. Antibody titers begin to rise approximately 7-9 days after infection and peak at 3-4 weeks. Enzyme immunoassay is more sensitive in detecting acute infection than culture and has sensitivity comparable to the polymerase chain reaction if there has been sufficient time to develop an antibody response.

Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and professor of dermatology and pediatrics at the University of California, San Diego. Dr. Bhatti is a research fellow in pediatric dermatology at Rady Children’s Hospital and the University of California, San Diego. They said they have no financial disclosures. Email Dr. Eichenfield and Dr. Bhatti at [email protected].

References

1. Arch Dermatol. 1993 Jan;129(1):92-6.

2. J Am Acad Dermatol. 2015 Feb;72(2):239-45.

About a third of peanut-allergic patients given oral immunotherapy (OIT) passed a peanut challenge when the therapy was reduced, based on data from a phase 2 randomized trial of individuals with confirmed peanut allergies.

yktr/ThinkStock

Previous studies have shown that desensitization to peanuts can be successful, but sustained response to oral immunotherapy after treatment reduction or discontinuation has not been well studied, wrote R. Sharon Chinthrajah, MD, of Stanford (Calif.)University, and colleagues.

“We found that OIT with peanut was able to desensitise people with peanut allergy to 4,000 mg of peanut protein, but that discontinuation of peanut, or even a reduction to 300 mg daily, increased the likelihood of regaining clinical reactivity to peanut,” they wrote. “With peanut allergy therapies in varying stages of clinical development, and some nearing [Food and Drug Administration] approval, vital questions remain regarding the durability of treatment effects and the appropriate maintenance doses.”

In the Peanut Oral Immunotherapy Study: Safety Efficacy and Discovery (POISED), published in The Lancet, the researchers randomized 120 participants to three groups:

• 60 patients built up to a maintenance dose of 4,000 mg of peanut protein for 104 weeks followed by total discontinuation (peanut-0).

• 35 patients built up to a maintenance dose of 4,000 mg of peanut protein for 104 weeks followed by a 300-mg maintenance dose of peanut protein in the form of peanut flour (peanut-300).

• 25 patients to an oat flour placebo.

All participants were trained on how and when to use epinephrine autoinjector devices to treat allergic symptoms such as respiratory problems (cough, shortness of breath, or change in voice), widespread hives or erythema, repetitive vomiting, persistent abdominal pain, angioedema of the face, or feeling faint.

The primary outcome was passing a double-blind, placebo-controlled, food challenge (DBPCFC) to 4,000 mg of peanut protein, which was measured at baseline and at weeks 104, 117, 130, 143, and 156.

SOURCE: Chinthrajah RS et al. Lancet. 2019 Sep 12. doi: 10.1016/S0140-6736(19)31793-3.

About a third of peanut-allergic patients given oral immunotherapy (OIT) passed a peanut challenge when the therapy was reduced, based on data from a phase 2 randomized trial of individuals with confirmed peanut allergies.

yktr/ThinkStock

Previous studies have shown that desensitization to peanuts can be successful, but sustained response to oral immunotherapy after treatment reduction or discontinuation has not been well studied, wrote R. Sharon Chinthrajah, MD, of Stanford (Calif.)University, and colleagues.

“We found that OIT with peanut was able to desensitise people with peanut allergy to 4,000 mg of peanut protein, but that discontinuation of peanut, or even a reduction to 300 mg daily, increased the likelihood of regaining clinical reactivity to peanut,” they wrote. “With peanut allergy therapies in varying stages of clinical development, and some nearing [Food and Drug Administration] approval, vital questions remain regarding the durability of treatment effects and the appropriate maintenance doses.”

In the Peanut Oral Immunotherapy Study: Safety Efficacy and Discovery (POISED), published in The Lancet, the researchers randomized 120 participants to three groups:

• 60 patients built up to a maintenance dose of 4,000 mg of peanut protein for 104 weeks followed by total discontinuation (peanut-0).

• 35 patients built up to a maintenance dose of 4,000 mg of peanut protein for 104 weeks followed by a 300-mg maintenance dose of peanut protein in the form of peanut flour (peanut-300).

• 25 patients to an oat flour placebo.

All participants were trained on how and when to use epinephrine autoinjector devices to treat allergic symptoms such as respiratory problems (cough, shortness of breath, or change in voice), widespread hives or erythema, repetitive vomiting, persistent abdominal pain, angioedema of the face, or feeling faint.

The primary outcome was passing a double-blind, placebo-controlled, food challenge (DBPCFC) to 4,000 mg of peanut protein, which was measured at baseline and at weeks 104, 117, 130, 143, and 156.

SOURCE: Chinthrajah RS et al. Lancet. 2019 Sep 12. doi: 10.1016/S0140-6736(19)31793-3.

About a third of peanut-allergic patients given oral immunotherapy (OIT) passed a peanut challenge when the therapy was reduced, based on data from a phase 2 randomized trial of individuals with confirmed peanut allergies.

yktr/ThinkStock

Previous studies have shown that desensitization to peanuts can be successful, but sustained response to oral immunotherapy after treatment reduction or discontinuation has not been well studied, wrote R. Sharon Chinthrajah, MD, of Stanford (Calif.)University, and colleagues.

“We found that OIT with peanut was able to desensitise people with peanut allergy to 4,000 mg of peanut protein, but that discontinuation of peanut, or even a reduction to 300 mg daily, increased the likelihood of regaining clinical reactivity to peanut,” they wrote. “With peanut allergy therapies in varying stages of clinical development, and some nearing [Food and Drug Administration] approval, vital questions remain regarding the durability of treatment effects and the appropriate maintenance doses.”

In the Peanut Oral Immunotherapy Study: Safety Efficacy and Discovery (POISED), published in The Lancet, the researchers randomized 120 participants to three groups:

• 60 patients built up to a maintenance dose of 4,000 mg of peanut protein for 104 weeks followed by total discontinuation (peanut-0).

• 35 patients built up to a maintenance dose of 4,000 mg of peanut protein for 104 weeks followed by a 300-mg maintenance dose of peanut protein in the form of peanut flour (peanut-300).

• 25 patients to an oat flour placebo.

All participants were trained on how and when to use epinephrine autoinjector devices to treat allergic symptoms such as respiratory problems (cough, shortness of breath, or change in voice), widespread hives or erythema, repetitive vomiting, persistent abdominal pain, angioedema of the face, or feeling faint.

The primary outcome was passing a double-blind, placebo-controlled, food challenge (DBPCFC) to 4,000 mg of peanut protein, which was measured at baseline and at weeks 104, 117, 130, 143, and 156.

SOURCE: Chinthrajah RS et al. Lancet. 2019 Sep 12. doi: 10.1016/S0140-6736(19)31793-3.

A 34-year-old woman self-refers to dermatology for evaluation of a very itchy rash that manifested 2 weeks ago on her right arm. She immediately went to an urgent care clinic, where she was diagnosed with shingles and prescribed valacyclovir. This diagnosis was upsetting to the patient, as she was advised to avoid contact with her newborn niece for at least 2 weeks.

Despite the prescribed medication, however, the rash began to pop up in other areas, including her left arm, chest, and face. Through all of this, the patient felt fine: no fever, myalgia, or malaise.

Her husband suggested she seek an appointment with dermatology, which was expedited by a phone call from her primary care provider.

EXAMINATION
The patient is afebrile and in no acute distress. She is, however, quite upset with the widespread collections of vesicles on mildly erythematous bases, many in a linear configuration. In several areas, there is ecchymosis secondary to scratching.

What’s the diagnosis?

DISCUSSION
Poison ivy, or Rhus dermatitis, is one of the most common dermatologic problems seen in medicine—and yet, its various presentations can, as this case illustrates, be quite confusing. Even when it is recognized, treatment is far from satisfactory (but more on that later). Furthermore, there is a lot of misinformation about everything from the appearance of the offending plant to the condition’s “contagious” nature.

From a broader perspective, poison ivy is becoming more prevalent and its effects more pronounced as cities expand into formerly open country. The Rhus plant family (Toxicodendron radicans and others) thrives on our increasing levels of CO₂, effectively making the “poisonous” resin in the stems, leaves, and berries more potent.

With repeated exposure, the vast majority of the population will develop an allergy to this resin, known as urushiol, which can persist even on long-dead plants, vines, and leaves. (It does take repeated exposure to develop the requisite T-cell population, which is why many children are immune to it.) The urushiol does not serve as a protective substance for the plant; rather, it helps the plant retain water. In fact, many animals feed on the plant with impunity.

Virtually all members of the poison ivy family display “leaves of three” emerging from a single stem, with each triplet alternating first on one side of the branch and then on the other. Several varieties of the plant flourish over vast areas of the world, but in the United States, east of the Rockies, Toxicodendron radicans is the dominant member of the family. It can grow as a low vine, a shrub, or a climbing vine, each with a distinct appearance aside from the leaves, which are almond-shaped, smooth, and usually shiny with smooth surfaces. Most mature leaves will have a single notch, sometimes called a “thumb,” on otherwise smooth, nonserrated edges. In the summer, tiny white and yellow berries begin to grow.

The climbing vines of older plants can reach heights of 10 meters or more. These vines can reach a thickness of 3 inches and often appear “furry,” with tiny rootlets covering their surfaces. Plants this large can produce leaves 12 to 14 inches long.

Clinically, the appearance of linear pink to red pruritic vesicular streaks typify this contact dermatitis, which can immediately follow exposure or take days to appear. Said exposure can be direct or via pets, tools, or aerosols (eg, from neighbors mowing their lawns). Besides avoidance of the great outdoors, washing thoroughly immediately after exposure makes sense (but many are unaware that they’ve been exposed until it’s too late).

Poison ivy is not contagious, though the general public firmly believes otherwise. Left untreated, it clears within 2 weeks (except in unusual cases). For those who cannot bear to wait, treatment is problematic, to say the least. OTC products, such as calamine lotion, do nothing for the itching but may help with blistering. Topical or systemic steroids reduce itching somewhat. Antihistamines are useless, since this condition does not involve histamine release.

TAKE-HOME LEARNING POINTS

• Poison ivy (Rhus dermatitis) is quite common and becoming more so due to encroaching civilization and increasing CO₂ levels.
• “Leaves of three, let it be” is still good advice, because the poison ivy plant Toxicodendron radicans manifests with three almond-shaped, shiny, green leaves grouped in threes.
• Urushiol is the name of the oily resin found in the plant’s stem, leaves, and berries, and is the trigger resulting in contact dermatitis.
• Poison ivy is not contagious and typically clears in 2 weeks.

A 34-year-old woman self-refers to dermatology for evaluation of a very itchy rash that manifested 2 weeks ago on her right arm. She immediately went to an urgent care clinic, where she was diagnosed with shingles and prescribed valacyclovir. This diagnosis was upsetting to the patient, as she was advised to avoid contact with her newborn niece for at least 2 weeks.

Despite the prescribed medication, however, the rash began to pop up in other areas, including her left arm, chest, and face. Through all of this, the patient felt fine: no fever, myalgia, or malaise.

Her husband suggested she seek an appointment with dermatology, which was expedited by a phone call from her primary care provider.

EXAMINATION
The patient is afebrile and in no acute distress. She is, however, quite upset with the widespread collections of vesicles on mildly erythematous bases, many in a linear configuration. In several areas, there is ecchymosis secondary to scratching.

What’s the diagnosis?

DISCUSSION
Poison ivy, or Rhus dermatitis, is one of the most common dermatologic problems seen in medicine—and yet, its various presentations can, as this case illustrates, be quite confusing. Even when it is recognized, treatment is far from satisfactory (but more on that later). Furthermore, there is a lot of misinformation about everything from the appearance of the offending plant to the condition’s “contagious” nature.

From a broader perspective, poison ivy is becoming more prevalent and its effects more pronounced as cities expand into formerly open country. The Rhus plant family (Toxicodendron radicans and others) thrives on our increasing levels of CO₂, effectively making the “poisonous” resin in the stems, leaves, and berries more potent.

With repeated exposure, the vast majority of the population will develop an allergy to this resin, known as urushiol, which can persist even on long-dead plants, vines, and leaves. (It does take repeated exposure to develop the requisite T-cell population, which is why many children are immune to it.) The urushiol does not serve as a protective substance for the plant; rather, it helps the plant retain water. In fact, many animals feed on the plant with impunity.

Virtually all members of the poison ivy family display “leaves of three” emerging from a single stem, with each triplet alternating first on one side of the branch and then on the other. Several varieties of the plant flourish over vast areas of the world, but in the United States, east of the Rockies, Toxicodendron radicans is the dominant member of the family. It can grow as a low vine, a shrub, or a climbing vine, each with a distinct appearance aside from the leaves, which are almond-shaped, smooth, and usually shiny with smooth surfaces. Most mature leaves will have a single notch, sometimes called a “thumb,” on otherwise smooth, nonserrated edges. In the summer, tiny white and yellow berries begin to grow.

The climbing vines of older plants can reach heights of 10 meters or more. These vines can reach a thickness of 3 inches and often appear “furry,” with tiny rootlets covering their surfaces. Plants this large can produce leaves 12 to 14 inches long.

Clinically, the appearance of linear pink to red pruritic vesicular streaks typify this contact dermatitis, which can immediately follow exposure or take days to appear. Said exposure can be direct or via pets, tools, or aerosols (eg, from neighbors mowing their lawns). Besides avoidance of the great outdoors, washing thoroughly immediately after exposure makes sense (but many are unaware that they’ve been exposed until it’s too late).

Poison ivy is not contagious, though the general public firmly believes otherwise. Left untreated, it clears within 2 weeks (except in unusual cases). For those who cannot bear to wait, treatment is problematic, to say the least. OTC products, such as calamine lotion, do nothing for the itching but may help with blistering. Topical or systemic steroids reduce itching somewhat. Antihistamines are useless, since this condition does not involve histamine release.

TAKE-HOME LEARNING POINTS

• Poison ivy (Rhus dermatitis) is quite common and becoming more so due to encroaching civilization and increasing CO₂ levels.
• “Leaves of three, let it be” is still good advice, because the poison ivy plant Toxicodendron radicans manifests with three almond-shaped, shiny, green leaves grouped in threes.
• Urushiol is the name of the oily resin found in the plant’s stem, leaves, and berries, and is the trigger resulting in contact dermatitis.
• Poison ivy is not contagious and typically clears in 2 weeks.

A 34-year-old woman self-refers to dermatology for evaluation of a very itchy rash that manifested 2 weeks ago on her right arm. She immediately went to an urgent care clinic, where she was diagnosed with shingles and prescribed valacyclovir. This diagnosis was upsetting to the patient, as she was advised to avoid contact with her newborn niece for at least 2 weeks.

Despite the prescribed medication, however, the rash began to pop up in other areas, including her left arm, chest, and face. Through all of this, the patient felt fine: no fever, myalgia, or malaise.

Her husband suggested she seek an appointment with dermatology, which was expedited by a phone call from her primary care provider.

EXAMINATION
The patient is afebrile and in no acute distress. She is, however, quite upset with the widespread collections of vesicles on mildly erythematous bases, many in a linear configuration. In several areas, there is ecchymosis secondary to scratching.

What’s the diagnosis?

DISCUSSION
Poison ivy, or Rhus dermatitis, is one of the most common dermatologic problems seen in medicine—and yet, its various presentations can, as this case illustrates, be quite confusing. Even when it is recognized, treatment is far from satisfactory (but more on that later). Furthermore, there is a lot of misinformation about everything from the appearance of the offending plant to the condition’s “contagious” nature.

From a broader perspective, poison ivy is becoming more prevalent and its effects more pronounced as cities expand into formerly open country. The Rhus plant family (Toxicodendron radicans and others) thrives on our increasing levels of CO₂, effectively making the “poisonous” resin in the stems, leaves, and berries more potent.

With repeated exposure, the vast majority of the population will develop an allergy to this resin, known as urushiol, which can persist even on long-dead plants, vines, and leaves. (It does take repeated exposure to develop the requisite T-cell population, which is why many children are immune to it.) The urushiol does not serve as a protective substance for the plant; rather, it helps the plant retain water. In fact, many animals feed on the plant with impunity.

Virtually all members of the poison ivy family display “leaves of three” emerging from a single stem, with each triplet alternating first on one side of the branch and then on the other. Several varieties of the plant flourish over vast areas of the world, but in the United States, east of the Rockies, Toxicodendron radicans is the dominant member of the family. It can grow as a low vine, a shrub, or a climbing vine, each with a distinct appearance aside from the leaves, which are almond-shaped, smooth, and usually shiny with smooth surfaces. Most mature leaves will have a single notch, sometimes called a “thumb,” on otherwise smooth, nonserrated edges. In the summer, tiny white and yellow berries begin to grow.

The climbing vines of older plants can reach heights of 10 meters or more. These vines can reach a thickness of 3 inches and often appear “furry,” with tiny rootlets covering their surfaces. Plants this large can produce leaves 12 to 14 inches long.

Clinically, the appearance of linear pink to red pruritic vesicular streaks typify this contact dermatitis, which can immediately follow exposure or take days to appear. Said exposure can be direct or via pets, tools, or aerosols (eg, from neighbors mowing their lawns). Besides avoidance of the great outdoors, washing thoroughly immediately after exposure makes sense (but many are unaware that they’ve been exposed until it’s too late).

Poison ivy is not contagious, though the general public firmly believes otherwise. Left untreated, it clears within 2 weeks (except in unusual cases). For those who cannot bear to wait, treatment is problematic, to say the least. OTC products, such as calamine lotion, do nothing for the itching but may help with blistering. Topical or systemic steroids reduce itching somewhat. Antihistamines are useless, since this condition does not involve histamine release.

TAKE-HOME LEARNING POINTS

• Poison ivy (Rhus dermatitis) is quite common and becoming more so due to encroaching civilization and increasing CO₂ levels.
• “Leaves of three, let it be” is still good advice, because the poison ivy plant Toxicodendron radicans manifests with three almond-shaped, shiny, green leaves grouped in threes.
• Urushiol is the name of the oily resin found in the plant’s stem, leaves, and berries, and is the trigger resulting in contact dermatitis.
• Poison ivy is not contagious and typically clears in 2 weeks.

The FP suspected cutaneous vasculitis of the ear caused by levamisole-adulterated cocaine.

Levamisole is an antihelminthic drug approved for veterinary purposes. In the past, the drug had been used as an immune modulator in autoimmune disorders, but no longer is considered safe for human use, as it can cause agranulocytosis. Sellers around the world often lace cocaine with levamisole because it boosts the profits and potentiates the psychoactive effects of the cocaine. Cutaneous vasculitis secondary to levamisole-adulterated cocaine has been reported many times in the literature.

Levamisole-associated vasculitis presents with ear purpura, retiform (like a net) purpura of the trunk or extremities, and neutropenia. Patients will test positive for perinuclear antineutrophil cytoplasmic antibody (pANCA). This cutaneous vasculitis also may present on the nose or face. There are reports of cocaine/levamisole-associated autoimmune syndrome involving agranulocytosis and cutaneous vasculitis.

The patient tested positive for pANCA, as was expected. The FP told her to discontinue her cocaine use, as she ran the risk of worse manifestations. She refused any treatment for her drug use and stated she could stop it on her own. The FP referred the patient to Dermatology, but the vasculitis was barely visible by the time she was seen. Convincing the patient not to use cocaine again remained the only treatment.

‌

Photo courtesy of Jon Karnes, MD, and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Mayeaux EJ, Usatine R, Martin N, et al. Vasculitis. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. Color Atlas and Synopsis of Family Medicine. 3rd ed. New York, NY: McGraw-Hill; 2019:1169-1173.

To learn more about the newest 3rd edition of the Color Atlas and Synopsis of Family Medicine, see: https://www.amazon.com/Color-Atlas-Synopsis-Family-Medicine/dp/1259862046/

You can get the 3rd edition of the Color Atlas and Synopsis of Family Medicine as an app by clicking on this link: https://usatinemedia.com/app/color-atlas-of-family-medicine/

The FP suspected cutaneous vasculitis of the ear caused by levamisole-adulterated cocaine.

Levamisole is an antihelminthic drug approved for veterinary purposes. In the past, the drug had been used as an immune modulator in autoimmune disorders, but no longer is considered safe for human use, as it can cause agranulocytosis. Sellers around the world often lace cocaine with levamisole because it boosts the profits and potentiates the psychoactive effects of the cocaine. Cutaneous vasculitis secondary to levamisole-adulterated cocaine has been reported many times in the literature.

Levamisole-associated vasculitis presents with ear purpura, retiform (like a net) purpura of the trunk or extremities, and neutropenia. Patients will test positive for perinuclear antineutrophil cytoplasmic antibody (pANCA). This cutaneous vasculitis also may present on the nose or face. There are reports of cocaine/levamisole-associated autoimmune syndrome involving agranulocytosis and cutaneous vasculitis.

The patient tested positive for pANCA, as was expected. The FP told her to discontinue her cocaine use, as she ran the risk of worse manifestations. She refused any treatment for her drug use and stated she could stop it on her own. The FP referred the patient to Dermatology, but the vasculitis was barely visible by the time she was seen. Convincing the patient not to use cocaine again remained the only treatment.

‌

Photo courtesy of Jon Karnes, MD, and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Mayeaux EJ, Usatine R, Martin N, et al. Vasculitis. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. Color Atlas and Synopsis of Family Medicine. 3rd ed. New York, NY: McGraw-Hill; 2019:1169-1173.

To learn more about the newest 3rd edition of the Color Atlas and Synopsis of Family Medicine, see: https://www.amazon.com/Color-Atlas-Synopsis-Family-Medicine/dp/1259862046/

You can get the 3rd edition of the Color Atlas and Synopsis of Family Medicine as an app by clicking on this link: https://usatinemedia.com/app/color-atlas-of-family-medicine/

The FP suspected cutaneous vasculitis of the ear caused by levamisole-adulterated cocaine.

Levamisole is an antihelminthic drug approved for veterinary purposes. In the past, the drug had been used as an immune modulator in autoimmune disorders, but no longer is considered safe for human use, as it can cause agranulocytosis. Sellers around the world often lace cocaine with levamisole because it boosts the profits and potentiates the psychoactive effects of the cocaine. Cutaneous vasculitis secondary to levamisole-adulterated cocaine has been reported many times in the literature.

Levamisole-associated vasculitis presents with ear purpura, retiform (like a net) purpura of the trunk or extremities, and neutropenia. Patients will test positive for perinuclear antineutrophil cytoplasmic antibody (pANCA). This cutaneous vasculitis also may present on the nose or face. There are reports of cocaine/levamisole-associated autoimmune syndrome involving agranulocytosis and cutaneous vasculitis.

The patient tested positive for pANCA, as was expected. The FP told her to discontinue her cocaine use, as she ran the risk of worse manifestations. She refused any treatment for her drug use and stated she could stop it on her own. The FP referred the patient to Dermatology, but the vasculitis was barely visible by the time she was seen. Convincing the patient not to use cocaine again remained the only treatment.

‌

Photo courtesy of Jon Karnes, MD, and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Mayeaux EJ, Usatine R, Martin N, et al. Vasculitis. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. Color Atlas and Synopsis of Family Medicine. 3rd ed. New York, NY: McGraw-Hill; 2019:1169-1173.

To learn more about the newest 3rd edition of the Color Atlas and Synopsis of Family Medicine, see: https://www.amazon.com/Color-Atlas-Synopsis-Family-Medicine/dp/1259862046/

You can get the 3rd edition of the Color Atlas and Synopsis of Family Medicine as an app by clicking on this link: https://usatinemedia.com/app/color-atlas-of-family-medicine/

AUSTIN, TEX. – If a child presents with acute photosensitivity at a young age, onset of freckling before the age of 2 years, and severe sun damage of the lips and eyes, think xeroderma pigmentosum (XP), a rare autosomal recessive disorder.

Doug Brunk/MDedge News

Other telltale symptoms of XP include the presence of skin cancer at an early age and a large number of skin cancers.

At the annual meeting of the Society for Pediatric Dermatology, John J. DiGiovanna, MD, described XP as a disorder of genomic instability, which has no cure. It’s caused by a mutation in genes XPA through XPG and the XP variant (XPV) gene. “The genome controls our genes, and UV rays damage DNA,” said Dr. DiGiovanna, who is a senior research physician at the National Cancer Institute’s Laboratory of Cancer and Biology and Genetics, Bethesda, Md. “This damage from UV radiation is similar to damage from chemical agents that form DNA adducts, such as cigarette smoke and certain chemotherapy agents such as cisplatinum.”

XP patients present with or without acute burning after minimal sun exposure, while children with both subtypes develop “freckling” by the time they reach 2 years of age. Dr. DiGiovanna pointed out that lentigo maligna lesions associated with XP resemble freckles at first glance, yet they vary in size, intensity, and border. Meanwhile, freckles in healthy patients are similar in size, are light tan in color, and have a regular border.

“The burning with minimal sun exposure that occurs during childhood leads to pigmentary changes, atrophy, xerosis, and telangiectasias,” he said. A follow-up analysis of 106 XP patients admitted to the National Institutes of Health between 1971 and 2009 found that patients were diagnosed with their first nonmelanoma skin cancer at a median age of 9 years, compared with age 67 among those in the general population (J Med Genet 2011 Mar;48[3]:168-76). “This is a 58-year decrease in age at risk, which is a 10,000-fold increase in skin cancer,” said Dr. DiGiovanna, who was one of the study authors.

Melanoma also occurs at an earlier age among XP patients – a median age of 22 years, compared with a median of 55 years in the general population. “In the general population, melanoma occurs at a younger age than nonmelanoma skin cancer, while in the XP population, melanoma occurs at an older age,” he said. “This is giving us a good biologic lesson that the melanoma induction mechanism must be different from nonmelanoma skin cancer.”

He recalled one XP patient who was followed by NIH researchers for 4 decades. She worked in a doctor’s office and drove a car, but developed progressive neurologic degeneration and died at the age of 40. “This was not due to unrepaired UV damage, but there are other agents which damage other neurons,” Dr. DiGiovanna explained. “Over time, what you get is a decrease in brain volume, an increase in the brain ventricles, and a loss of brain tissue. At postmortem examination, her brain was of infantile size, compared with that of an equivalent 40-year-old. This is a disease of neuronal loss, and it’s progressive. Only about 20%-25% of XP patients experience neural degeneration.”

Management of XP involves strict sun avoidance, including use of a portable UV meter and many layers of UV protection, including application of sunscreen, wearing protective clothing, sunglasses, hats, and face shields, and the use of UV-blocking window film, LED lights, and a vitamin D diet or oral supplementation. Affected individuals also require frequent skin monitoring by the patients and their family members, frequent dermatologic exams by clinicians, biopsy of suspicious lesions, removal of any skin cancers found, field treatments with agents such as 5-fluorouracil and imiquimod, and chemoprevention with oral retinoids for patients who are actively developing large numbers of new lesions (N Engl J Med. 1988 Jun23;318[25]:1633-7).

“Probably the most important thing you can do is refer them to patient support groups,” Dr. DiGiovanna said. “They are present in many countries and can help them manage the day-to-day issues of their condition.” Support groups based in North America include the XP Family Support Group, XP Society, and XP Grupo Luz De Esperanza.

Dr. DiGiovanna reported having no financial disclosures.

[email protected]

AUSTIN, TEX. – If a child presents with acute photosensitivity at a young age, onset of freckling before the age of 2 years, and severe sun damage of the lips and eyes, think xeroderma pigmentosum (XP), a rare autosomal recessive disorder.

Doug Brunk/MDedge News

Other telltale symptoms of XP include the presence of skin cancer at an early age and a large number of skin cancers.

At the annual meeting of the Society for Pediatric Dermatology, John J. DiGiovanna, MD, described XP as a disorder of genomic instability, which has no cure. It’s caused by a mutation in genes XPA through XPG and the XP variant (XPV) gene. “The genome controls our genes, and UV rays damage DNA,” said Dr. DiGiovanna, who is a senior research physician at the National Cancer Institute’s Laboratory of Cancer and Biology and Genetics, Bethesda, Md. “This damage from UV radiation is similar to damage from chemical agents that form DNA adducts, such as cigarette smoke and certain chemotherapy agents such as cisplatinum.”

XP patients present with or without acute burning after minimal sun exposure, while children with both subtypes develop “freckling” by the time they reach 2 years of age. Dr. DiGiovanna pointed out that lentigo maligna lesions associated with XP resemble freckles at first glance, yet they vary in size, intensity, and border. Meanwhile, freckles in healthy patients are similar in size, are light tan in color, and have a regular border.

“The burning with minimal sun exposure that occurs during childhood leads to pigmentary changes, atrophy, xerosis, and telangiectasias,” he said. A follow-up analysis of 106 XP patients admitted to the National Institutes of Health between 1971 and 2009 found that patients were diagnosed with their first nonmelanoma skin cancer at a median age of 9 years, compared with age 67 among those in the general population (J Med Genet 2011 Mar;48[3]:168-76). “This is a 58-year decrease in age at risk, which is a 10,000-fold increase in skin cancer,” said Dr. DiGiovanna, who was one of the study authors.

Melanoma also occurs at an earlier age among XP patients – a median age of 22 years, compared with a median of 55 years in the general population. “In the general population, melanoma occurs at a younger age than nonmelanoma skin cancer, while in the XP population, melanoma occurs at an older age,” he said. “This is giving us a good biologic lesson that the melanoma induction mechanism must be different from nonmelanoma skin cancer.”

He recalled one XP patient who was followed by NIH researchers for 4 decades. She worked in a doctor’s office and drove a car, but developed progressive neurologic degeneration and died at the age of 40. “This was not due to unrepaired UV damage, but there are other agents which damage other neurons,” Dr. DiGiovanna explained. “Over time, what you get is a decrease in brain volume, an increase in the brain ventricles, and a loss of brain tissue. At postmortem examination, her brain was of infantile size, compared with that of an equivalent 40-year-old. This is a disease of neuronal loss, and it’s progressive. Only about 20%-25% of XP patients experience neural degeneration.”

Management of XP involves strict sun avoidance, including use of a portable UV meter and many layers of UV protection, including application of sunscreen, wearing protective clothing, sunglasses, hats, and face shields, and the use of UV-blocking window film, LED lights, and a vitamin D diet or oral supplementation. Affected individuals also require frequent skin monitoring by the patients and their family members, frequent dermatologic exams by clinicians, biopsy of suspicious lesions, removal of any skin cancers found, field treatments with agents such as 5-fluorouracil and imiquimod, and chemoprevention with oral retinoids for patients who are actively developing large numbers of new lesions (N Engl J Med. 1988 Jun23;318[25]:1633-7).

“Probably the most important thing you can do is refer them to patient support groups,” Dr. DiGiovanna said. “They are present in many countries and can help them manage the day-to-day issues of their condition.” Support groups based in North America include the XP Family Support Group, XP Society, and XP Grupo Luz De Esperanza.

Dr. DiGiovanna reported having no financial disclosures.

[email protected]

AUSTIN, TEX. – If a child presents with acute photosensitivity at a young age, onset of freckling before the age of 2 years, and severe sun damage of the lips and eyes, think xeroderma pigmentosum (XP), a rare autosomal recessive disorder.

Doug Brunk/MDedge News

Other telltale symptoms of XP include the presence of skin cancer at an early age and a large number of skin cancers.

At the annual meeting of the Society for Pediatric Dermatology, John J. DiGiovanna, MD, described XP as a disorder of genomic instability, which has no cure. It’s caused by a mutation in genes XPA through XPG and the XP variant (XPV) gene. “The genome controls our genes, and UV rays damage DNA,” said Dr. DiGiovanna, who is a senior research physician at the National Cancer Institute’s Laboratory of Cancer and Biology and Genetics, Bethesda, Md. “This damage from UV radiation is similar to damage from chemical agents that form DNA adducts, such as cigarette smoke and certain chemotherapy agents such as cisplatinum.”

XP patients present with or without acute burning after minimal sun exposure, while children with both subtypes develop “freckling” by the time they reach 2 years of age. Dr. DiGiovanna pointed out that lentigo maligna lesions associated with XP resemble freckles at first glance, yet they vary in size, intensity, and border. Meanwhile, freckles in healthy patients are similar in size, are light tan in color, and have a regular border.

“The burning with minimal sun exposure that occurs during childhood leads to pigmentary changes, atrophy, xerosis, and telangiectasias,” he said. A follow-up analysis of 106 XP patients admitted to the National Institutes of Health between 1971 and 2009 found that patients were diagnosed with their first nonmelanoma skin cancer at a median age of 9 years, compared with age 67 among those in the general population (J Med Genet 2011 Mar;48[3]:168-76). “This is a 58-year decrease in age at risk, which is a 10,000-fold increase in skin cancer,” said Dr. DiGiovanna, who was one of the study authors.

Melanoma also occurs at an earlier age among XP patients – a median age of 22 years, compared with a median of 55 years in the general population. “In the general population, melanoma occurs at a younger age than nonmelanoma skin cancer, while in the XP population, melanoma occurs at an older age,” he said. “This is giving us a good biologic lesson that the melanoma induction mechanism must be different from nonmelanoma skin cancer.”

He recalled one XP patient who was followed by NIH researchers for 4 decades. She worked in a doctor’s office and drove a car, but developed progressive neurologic degeneration and died at the age of 40. “This was not due to unrepaired UV damage, but there are other agents which damage other neurons,” Dr. DiGiovanna explained. “Over time, what you get is a decrease in brain volume, an increase in the brain ventricles, and a loss of brain tissue. At postmortem examination, her brain was of infantile size, compared with that of an equivalent 40-year-old. This is a disease of neuronal loss, and it’s progressive. Only about 20%-25% of XP patients experience neural degeneration.”

Management of XP involves strict sun avoidance, including use of a portable UV meter and many layers of UV protection, including application of sunscreen, wearing protective clothing, sunglasses, hats, and face shields, and the use of UV-blocking window film, LED lights, and a vitamin D diet or oral supplementation. Affected individuals also require frequent skin monitoring by the patients and their family members, frequent dermatologic exams by clinicians, biopsy of suspicious lesions, removal of any skin cancers found, field treatments with agents such as 5-fluorouracil and imiquimod, and chemoprevention with oral retinoids for patients who are actively developing large numbers of new lesions (N Engl J Med. 1988 Jun23;318[25]:1633-7).

“Probably the most important thing you can do is refer them to patient support groups,” Dr. DiGiovanna said. “They are present in many countries and can help them manage the day-to-day issues of their condition.” Support groups based in North America include the XP Family Support Group, XP Society, and XP Grupo Luz De Esperanza.

Dr. DiGiovanna reported having no financial disclosures.

[email protected]

Young people who have received allogeneic hematopoietic stem cell transplants (HSCTs) are more likely to wear hats, sunscreen and other sun protection, but still intentionally tan and experience sunburn at the same rate as their peers, new research suggests.

Yuri Arcurs/Fotolia

In a survey‐based, cross‐sectional cohort study, researchers compared sun-protection behaviors and sun exposure in 85 children aged 21 years and younger who had undergone HSCT and 85 age-, sex-, and skin type–matched controls. The findings were published in Pediatric Dermatology.

HSCT recipients have a higher risk of long-term complications such as skin cancer, for which sun exposure is a major modifiable environmental risk factor.

“Therefore, consistent sun avoidance and protection as well as regular dermatologic evaluations are important for HSCT recipients,” wrote Edward B. Li, PhD, from Harvard Medical School, Boston, and coauthors.

The survey found no significant difference between the transplant and control group in the amount of intentional sun exposure, such as the amount of time spent outside on weekdays and weekends during the peak sun intensity hours of 10 a.m. and 4 p.m. More than one in five transplant recipients (21.2%) reported spending at least 3 hours a day outside between 10 a.m. and 4 p.m. on weekdays, as did 36.5% of transplant recipients on weekends.

There were also no significant differences between the two groups in terms of time spent tanning, either in the sun or in a tanning bed. Additionally, a similar number of transplant recipients and controls experienced one or more red or painful sunburns in the past year (25.9% vs. 27.1%).

However, transplant patients did practice better sun protection behaviors than did the control group, with 60% reporting that they always wore sunscreen, compared with 29.4% of controls. The transplant recipients were also significantly more likely to wear sunglasses and a hat and to stay in the shade or use an umbrella.

“While these data may reflect that HSCT patients are not practicing adequate sun avoidance, it may also suggest that these long‐term survivors are able to enjoy being outdoors as much as their peers and have a similar desire to have a tanned appearance,” the researchers wrote. “While a healthy and active lifestyle should be encouraged for all children, our results emphasize the need for pediatric HSCT survivors to be educated on their increased risk for UV‐related skin cancers, counseled on avoidance of intentional tanning, and advised on the importance of sun protection behaviors in an effort to improve long-term outcomes.”

The researchers noted that transplant recipients were significantly more likely to have had a full body skin exam from a health care professional than were individuals in the control group (61.2% vs. 4.7%) and were more likely to have done a self-check or been checked by a partner in the previous year.

The study was supported by the Society for Pediatric Dermatology, the Dermatology Foundation, the National Institutes of Health, and the Dr. Miriam and Sheldon G. Adelson Medical Research Foundation. One author declared a financial interest in a company developing a dermatological product. No other conflicts of interest were declared.

SOURCE: Li EB et al. Pediatr Dermatol. 2019 Aug 13. doi: 10.1111/pde.13984.

Young people who have received allogeneic hematopoietic stem cell transplants (HSCTs) are more likely to wear hats, sunscreen and other sun protection, but still intentionally tan and experience sunburn at the same rate as their peers, new research suggests.

Yuri Arcurs/Fotolia

In a survey‐based, cross‐sectional cohort study, researchers compared sun-protection behaviors and sun exposure in 85 children aged 21 years and younger who had undergone HSCT and 85 age-, sex-, and skin type–matched controls. The findings were published in Pediatric Dermatology.

HSCT recipients have a higher risk of long-term complications such as skin cancer, for which sun exposure is a major modifiable environmental risk factor.

“Therefore, consistent sun avoidance and protection as well as regular dermatologic evaluations are important for HSCT recipients,” wrote Edward B. Li, PhD, from Harvard Medical School, Boston, and coauthors.

The survey found no significant difference between the transplant and control group in the amount of intentional sun exposure, such as the amount of time spent outside on weekdays and weekends during the peak sun intensity hours of 10 a.m. and 4 p.m. More than one in five transplant recipients (21.2%) reported spending at least 3 hours a day outside between 10 a.m. and 4 p.m. on weekdays, as did 36.5% of transplant recipients on weekends.

There were also no significant differences between the two groups in terms of time spent tanning, either in the sun or in a tanning bed. Additionally, a similar number of transplant recipients and controls experienced one or more red or painful sunburns in the past year (25.9% vs. 27.1%).

However, transplant patients did practice better sun protection behaviors than did the control group, with 60% reporting that they always wore sunscreen, compared with 29.4% of controls. The transplant recipients were also significantly more likely to wear sunglasses and a hat and to stay in the shade or use an umbrella.

“While these data may reflect that HSCT patients are not practicing adequate sun avoidance, it may also suggest that these long‐term survivors are able to enjoy being outdoors as much as their peers and have a similar desire to have a tanned appearance,” the researchers wrote. “While a healthy and active lifestyle should be encouraged for all children, our results emphasize the need for pediatric HSCT survivors to be educated on their increased risk for UV‐related skin cancers, counseled on avoidance of intentional tanning, and advised on the importance of sun protection behaviors in an effort to improve long-term outcomes.”

The researchers noted that transplant recipients were significantly more likely to have had a full body skin exam from a health care professional than were individuals in the control group (61.2% vs. 4.7%) and were more likely to have done a self-check or been checked by a partner in the previous year.

The study was supported by the Society for Pediatric Dermatology, the Dermatology Foundation, the National Institutes of Health, and the Dr. Miriam and Sheldon G. Adelson Medical Research Foundation. One author declared a financial interest in a company developing a dermatological product. No other conflicts of interest were declared.

SOURCE: Li EB et al. Pediatr Dermatol. 2019 Aug 13. doi: 10.1111/pde.13984.

Young people who have received allogeneic hematopoietic stem cell transplants (HSCTs) are more likely to wear hats, sunscreen and other sun protection, but still intentionally tan and experience sunburn at the same rate as their peers, new research suggests.

Yuri Arcurs/Fotolia

In a survey‐based, cross‐sectional cohort study, researchers compared sun-protection behaviors and sun exposure in 85 children aged 21 years and younger who had undergone HSCT and 85 age-, sex-, and skin type–matched controls. The findings were published in Pediatric Dermatology.

HSCT recipients have a higher risk of long-term complications such as skin cancer, for which sun exposure is a major modifiable environmental risk factor.

“Therefore, consistent sun avoidance and protection as well as regular dermatologic evaluations are important for HSCT recipients,” wrote Edward B. Li, PhD, from Harvard Medical School, Boston, and coauthors.

The survey found no significant difference between the transplant and control group in the amount of intentional sun exposure, such as the amount of time spent outside on weekdays and weekends during the peak sun intensity hours of 10 a.m. and 4 p.m. More than one in five transplant recipients (21.2%) reported spending at least 3 hours a day outside between 10 a.m. and 4 p.m. on weekdays, as did 36.5% of transplant recipients on weekends.

There were also no significant differences between the two groups in terms of time spent tanning, either in the sun or in a tanning bed. Additionally, a similar number of transplant recipients and controls experienced one or more red or painful sunburns in the past year (25.9% vs. 27.1%).

However, transplant patients did practice better sun protection behaviors than did the control group, with 60% reporting that they always wore sunscreen, compared with 29.4% of controls. The transplant recipients were also significantly more likely to wear sunglasses and a hat and to stay in the shade or use an umbrella.

“While these data may reflect that HSCT patients are not practicing adequate sun avoidance, it may also suggest that these long‐term survivors are able to enjoy being outdoors as much as their peers and have a similar desire to have a tanned appearance,” the researchers wrote. “While a healthy and active lifestyle should be encouraged for all children, our results emphasize the need for pediatric HSCT survivors to be educated on their increased risk for UV‐related skin cancers, counseled on avoidance of intentional tanning, and advised on the importance of sun protection behaviors in an effort to improve long-term outcomes.”

The researchers noted that transplant recipients were significantly more likely to have had a full body skin exam from a health care professional than were individuals in the control group (61.2% vs. 4.7%) and were more likely to have done a self-check or been checked by a partner in the previous year.

The study was supported by the Society for Pediatric Dermatology, the Dermatology Foundation, the National Institutes of Health, and the Dr. Miriam and Sheldon G. Adelson Medical Research Foundation. One author declared a financial interest in a company developing a dermatological product. No other conflicts of interest were declared.

SOURCE: Li EB et al. Pediatr Dermatol. 2019 Aug 13. doi: 10.1111/pde.13984.

Immune dysregulation is a substantial feature of hidradenitis suppurativa in African American patients, according to data from 16 adults.

The etiology of hidradenitis suppurativa (HS) remains unknown, but neutrophils are prominent in affected areas of skin, wrote Angel S. Byrd, MD, PhD, of Johns Hopkins University, Baltimore, and colleagues.

“Among the several functions of neutrophils, these cells have the ability to form neutrophil extracellular traps (NETs) after exposure to certain microbes or sterile stimuli,” the researchers noted. These NETs are weblike structures that have been shown to activate several types of immune responses and promote inflammation.

In a study published in Science Translational Medicine, the researchers analyzed biospecimens from African American HS patients. They determined that NET structures were present in the lesional skin of HS patients, but not in control skin. Additionally, serum from HS patients did not properly degrade healthy control NETs in an in vitro examination.

A significant correlation (P less than .0001) occurred between the amount of NETs released in hidradenitis suppurativa lesions and hidradenitis suppurativa severity.

“Together, these results indicate that NET formation is enhanced in HS, both systemically and in lesional skin, in association with disease severity and that NET degradation mechanisms may be impaired in this disease,” the researchers wrote.

The researchers also found that total immunoglobulin G was significantly increased in the sera of HS patients, compared with that of healthy volunteers (P = .0011), and that enzymes involved in skin citrullination were elevated in the skin of HS patients, compared with controls’ skin.

Previous studies have shown that NETs can promote type I interferon (IFN) signatures in the blood of patients with autoimmune and chronic inflammatory conditions, and in this study, several type I IFN–regulated genes had increased expression in HS skin, including IFI44L, MX1, CXCL10, RSAD2, and IFI27.

The study findings were limited by the small sample size and homogenous population, the researchers noted. Future research should include “the role that neutrophil/type I IFN dysregulation plays in the clinical manifestations of the disease, and how these abnormalities in immune responses regulate other innate and adaptive immune cell types in HS,” which could affect the development of new treatments, they wrote.

The study was supported in part by the National Institutes of Health, the Johns Hopkins School of Medicine Physician Scientist Training Program, and the Danby Hidradenitis Suppurativa Foundation. Dr. Byrd disclosed participating in the Johns Hopkins Ethnic Skin Fellowship, which was supported in part by Valeant. Dr. Byrd also disclosed serving as a former investigator for Eli Lilly.

SOURCE: Byrd AS et al. Sci Transl Med. 2019 Sep 4. doi: 10.1126/scitranslmed.aav5908.

Immune dysregulation is a substantial feature of hidradenitis suppurativa in African American patients, according to data from 16 adults.

The etiology of hidradenitis suppurativa (HS) remains unknown, but neutrophils are prominent in affected areas of skin, wrote Angel S. Byrd, MD, PhD, of Johns Hopkins University, Baltimore, and colleagues.

“Among the several functions of neutrophils, these cells have the ability to form neutrophil extracellular traps (NETs) after exposure to certain microbes or sterile stimuli,” the researchers noted. These NETs are weblike structures that have been shown to activate several types of immune responses and promote inflammation.

In a study published in Science Translational Medicine, the researchers analyzed biospecimens from African American HS patients. They determined that NET structures were present in the lesional skin of HS patients, but not in control skin. Additionally, serum from HS patients did not properly degrade healthy control NETs in an in vitro examination.

A significant correlation (P less than .0001) occurred between the amount of NETs released in hidradenitis suppurativa lesions and hidradenitis suppurativa severity.

“Together, these results indicate that NET formation is enhanced in HS, both systemically and in lesional skin, in association with disease severity and that NET degradation mechanisms may be impaired in this disease,” the researchers wrote.

The researchers also found that total immunoglobulin G was significantly increased in the sera of HS patients, compared with that of healthy volunteers (P = .0011), and that enzymes involved in skin citrullination were elevated in the skin of HS patients, compared with controls’ skin.

Previous studies have shown that NETs can promote type I interferon (IFN) signatures in the blood of patients with autoimmune and chronic inflammatory conditions, and in this study, several type I IFN–regulated genes had increased expression in HS skin, including IFI44L, MX1, CXCL10, RSAD2, and IFI27.

The study findings were limited by the small sample size and homogenous population, the researchers noted. Future research should include “the role that neutrophil/type I IFN dysregulation plays in the clinical manifestations of the disease, and how these abnormalities in immune responses regulate other innate and adaptive immune cell types in HS,” which could affect the development of new treatments, they wrote.

The study was supported in part by the National Institutes of Health, the Johns Hopkins School of Medicine Physician Scientist Training Program, and the Danby Hidradenitis Suppurativa Foundation. Dr. Byrd disclosed participating in the Johns Hopkins Ethnic Skin Fellowship, which was supported in part by Valeant. Dr. Byrd also disclosed serving as a former investigator for Eli Lilly.

SOURCE: Byrd AS et al. Sci Transl Med. 2019 Sep 4. doi: 10.1126/scitranslmed.aav5908.

Immune dysregulation is a substantial feature of hidradenitis suppurativa in African American patients, according to data from 16 adults.

The etiology of hidradenitis suppurativa (HS) remains unknown, but neutrophils are prominent in affected areas of skin, wrote Angel S. Byrd, MD, PhD, of Johns Hopkins University, Baltimore, and colleagues.

“Among the several functions of neutrophils, these cells have the ability to form neutrophil extracellular traps (NETs) after exposure to certain microbes or sterile stimuli,” the researchers noted. These NETs are weblike structures that have been shown to activate several types of immune responses and promote inflammation.

In a study published in Science Translational Medicine, the researchers analyzed biospecimens from African American HS patients. They determined that NET structures were present in the lesional skin of HS patients, but not in control skin. Additionally, serum from HS patients did not properly degrade healthy control NETs in an in vitro examination.

A significant correlation (P less than .0001) occurred between the amount of NETs released in hidradenitis suppurativa lesions and hidradenitis suppurativa severity.

“Together, these results indicate that NET formation is enhanced in HS, both systemically and in lesional skin, in association with disease severity and that NET degradation mechanisms may be impaired in this disease,” the researchers wrote.

The researchers also found that total immunoglobulin G was significantly increased in the sera of HS patients, compared with that of healthy volunteers (P = .0011), and that enzymes involved in skin citrullination were elevated in the skin of HS patients, compared with controls’ skin.

Previous studies have shown that NETs can promote type I interferon (IFN) signatures in the blood of patients with autoimmune and chronic inflammatory conditions, and in this study, several type I IFN–regulated genes had increased expression in HS skin, including IFI44L, MX1, CXCL10, RSAD2, and IFI27.

The study findings were limited by the small sample size and homogenous population, the researchers noted. Future research should include “the role that neutrophil/type I IFN dysregulation plays in the clinical manifestations of the disease, and how these abnormalities in immune responses regulate other innate and adaptive immune cell types in HS,” which could affect the development of new treatments, they wrote.

The study was supported in part by the National Institutes of Health, the Johns Hopkins School of Medicine Physician Scientist Training Program, and the Danby Hidradenitis Suppurativa Foundation. Dr. Byrd disclosed participating in the Johns Hopkins Ethnic Skin Fellowship, which was supported in part by Valeant. Dr. Byrd also disclosed serving as a former investigator for Eli Lilly.

SOURCE: Byrd AS et al. Sci Transl Med. 2019 Sep 4. doi: 10.1126/scitranslmed.aav5908.

A 21-year-old woman presented with a rash on her right thigh of 3 to 4 months’ duration. She reported that the patch was asymptomatic. She was not taking any medications and otherwise was in good health. A review of systems was negative. The patient was a student who used her laptop frequently. On physical examination, a 10×5-cm reticulated, hyperpigmented patch was seen on her right thigh (FIGURE).

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

Erythema ab igne (EAI) is a common dermatosis caused by repeated exposure to infrared radiation, most commonly in the form of low-grade heat (43–47°C).¹ Common heat sources include heating pads, heaters, fire, and battery-charged devices. The distribution of the rash is dependent on the location of the heat source and appears as a hyperpigmented, reticulated rash. The pathophysiology is not well understood, but likely involves changes in dermal elastic fibers as well as the dermal venous plexus.² Though rare, chronic cases of EAI have been associated with cutaneous dysplasia.³

Inquire about possible heat sources in cases of suspected erythema ab igne.

Diagnosis of EAI is made by a combination of medical history and clinical features. Laboratory tests are not required. Additionally, clinicians should inquire about possible heat sources. In this case, we asked the patient whether she rested anything on her thighs, and she acknowledged that this was where she typically placed her laptop computer.

Differential includes other reticulated conditions

The differential diagnosis of a reticulated patch includes other entities likely sharing vascular pathology. The age, sex, and medical history of the patient offer additional diagnostic clues.

Livedo reticularis presents with reticulated erythema. It is unrelated to heat exposure, but may be associated with cold exposure. It can be physiologic or can be associated with vasculitis or another obstruction of blood flow.

Erythema infectiosum is a parvovirus B19 infection that usually presents in young children. It often results in a lacy reticulated exanthem on the face that resembles a slapped cheek in children. Adolescent and adult contacts often present with a more petechial rash in an acral to periflexural distribution.⁴

Continue to: Polyarteritis nodosa

Polyarteritis nodosa is a rare necrotizing vasculitis of small and medium arteries with an incidence of 4 to 16 cases per million.⁴ It usually is painful and can present with nodules, ulcers, or bullae and may be associated with livedo-like reticulated pigmentation.

Livedoid vasculitis is a hyalinization of blood vessels leading to the obstruction of vessels due to a hypercoagulable state. It can be acquired or congenital and usually manifests in middle-aged women.⁴

Management is straight-forward: Remove the heat source

EAI typically is asymptomatic, although there are reports of mild pruritus or a burning sensation. Management includes withdrawal of the heat source and patient education. Our patient’s rash went away when she stopped resting her laptop computer on her lap.

CORRESPONDENCE
Lorraine C. Young, MD, 200 UCLA, Medical Plaza Driveway, Suites 450 & 465, Los Angeles, CA 90095; [email protected]

A 21-year-old woman presented with a rash on her right thigh of 3 to 4 months’ duration. She reported that the patch was asymptomatic. She was not taking any medications and otherwise was in good health. A review of systems was negative. The patient was a student who used her laptop frequently. On physical examination, a 10×5-cm reticulated, hyperpigmented patch was seen on her right thigh (FIGURE).

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

Erythema ab igne (EAI) is a common dermatosis caused by repeated exposure to infrared radiation, most commonly in the form of low-grade heat (43–47°C).¹ Common heat sources include heating pads, heaters, fire, and battery-charged devices. The distribution of the rash is dependent on the location of the heat source and appears as a hyperpigmented, reticulated rash. The pathophysiology is not well understood, but likely involves changes in dermal elastic fibers as well as the dermal venous plexus.² Though rare, chronic cases of EAI have been associated with cutaneous dysplasia.³

Inquire about possible heat sources in cases of suspected erythema ab igne.

Diagnosis of EAI is made by a combination of medical history and clinical features. Laboratory tests are not required. Additionally, clinicians should inquire about possible heat sources. In this case, we asked the patient whether she rested anything on her thighs, and she acknowledged that this was where she typically placed her laptop computer.

Differential includes other reticulated conditions

The differential diagnosis of a reticulated patch includes other entities likely sharing vascular pathology. The age, sex, and medical history of the patient offer additional diagnostic clues.

Livedo reticularis presents with reticulated erythema. It is unrelated to heat exposure, but may be associated with cold exposure. It can be physiologic or can be associated with vasculitis or another obstruction of blood flow.

Erythema infectiosum is a parvovirus B19 infection that usually presents in young children. It often results in a lacy reticulated exanthem on the face that resembles a slapped cheek in children. Adolescent and adult contacts often present with a more petechial rash in an acral to periflexural distribution.⁴

Continue to: Polyarteritis nodosa

Polyarteritis nodosa is a rare necrotizing vasculitis of small and medium arteries with an incidence of 4 to 16 cases per million.⁴ It usually is painful and can present with nodules, ulcers, or bullae and may be associated with livedo-like reticulated pigmentation.

Livedoid vasculitis is a hyalinization of blood vessels leading to the obstruction of vessels due to a hypercoagulable state. It can be acquired or congenital and usually manifests in middle-aged women.⁴

Management is straight-forward: Remove the heat source

EAI typically is asymptomatic, although there are reports of mild pruritus or a burning sensation. Management includes withdrawal of the heat source and patient education. Our patient’s rash went away when she stopped resting her laptop computer on her lap.

CORRESPONDENCE
Lorraine C. Young, MD, 200 UCLA, Medical Plaza Driveway, Suites 450 & 465, Los Angeles, CA 90095; [email protected]

A 21-year-old woman presented with a rash on her right thigh of 3 to 4 months’ duration. She reported that the patch was asymptomatic. She was not taking any medications and otherwise was in good health. A review of systems was negative. The patient was a student who used her laptop frequently. On physical examination, a 10×5-cm reticulated, hyperpigmented patch was seen on her right thigh (FIGURE).

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

Erythema ab igne (EAI) is a common dermatosis caused by repeated exposure to infrared radiation, most commonly in the form of low-grade heat (43–47°C).¹ Common heat sources include heating pads, heaters, fire, and battery-charged devices. The distribution of the rash is dependent on the location of the heat source and appears as a hyperpigmented, reticulated rash. The pathophysiology is not well understood, but likely involves changes in dermal elastic fibers as well as the dermal venous plexus.² Though rare, chronic cases of EAI have been associated with cutaneous dysplasia.³

Inquire about possible heat sources in cases of suspected erythema ab igne.

Diagnosis of EAI is made by a combination of medical history and clinical features. Laboratory tests are not required. Additionally, clinicians should inquire about possible heat sources. In this case, we asked the patient whether she rested anything on her thighs, and she acknowledged that this was where she typically placed her laptop computer.

Differential includes other reticulated conditions

The differential diagnosis of a reticulated patch includes other entities likely sharing vascular pathology. The age, sex, and medical history of the patient offer additional diagnostic clues.

Livedo reticularis presents with reticulated erythema. It is unrelated to heat exposure, but may be associated with cold exposure. It can be physiologic or can be associated with vasculitis or another obstruction of blood flow.

Erythema infectiosum is a parvovirus B19 infection that usually presents in young children. It often results in a lacy reticulated exanthem on the face that resembles a slapped cheek in children. Adolescent and adult contacts often present with a more petechial rash in an acral to periflexural distribution.⁴

Continue to: Polyarteritis nodosa

Polyarteritis nodosa is a rare necrotizing vasculitis of small and medium arteries with an incidence of 4 to 16 cases per million.⁴ It usually is painful and can present with nodules, ulcers, or bullae and may be associated with livedo-like reticulated pigmentation.

Livedoid vasculitis is a hyalinization of blood vessels leading to the obstruction of vessels due to a hypercoagulable state. It can be acquired or congenital and usually manifests in middle-aged women.⁴

Management is straight-forward: Remove the heat source

EAI typically is asymptomatic, although there are reports of mild pruritus or a burning sensation. Management includes withdrawal of the heat source and patient education. Our patient’s rash went away when she stopped resting her laptop computer on her lap.

CORRESPONDENCE
Lorraine C. Young, MD, 200 UCLA, Medical Plaza Driveway, Suites 450 & 465, Los Angeles, CA 90095; [email protected]

The FP suspected acute systemic lupus erythematosus (SLE) with an acute cutaneous component. Laboratory testing showed a very high level of antinuclear antibodies. The patient was referred to Rheumatology and Dermatology. The dermatologist was available for a phone consult and suggested starting the patient on prednisone 60 mg/d as the Medrol Dosepak that the patient previously received had insufficient prednisolone for this severe flare of acute cutaneous lupus.

Although the classic description of acute SLE involves a butterfly rash, the rash of acute cutaneous lupus can include other areas of the face and body. As was seen in this case, the nasolabial fold tends to be spared and there are often skin erosions and crusting.

Based on the patient’s lab tests and symptoms, the dermatologist determined that the patient met the criteria for SLE. The patient was started on hydroxychloroquine 400 mg/d. The plan was to taper the patient’s prednisone slowly. By the following week, her skin and fatigue were much improved.

‌

Photos and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Pye A, Mayeaux EJ, Mishra V, et al. Lupus. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. Color Atlas and Synopsis of Family Medicine. 3rd ed. New York, NY: McGraw-Hill; 2019:1183-1193.

To learn more about the newest 3rd edition of the Color Atlas and Synopsis of Family Medicine, see: https://www.amazon.com/Color-Atlas-Synopsis-Family-Medicine/dp/1259862046/

You can get the 3rd edition of the Color Atlas and Synopsis of Family Medicine as an app by clicking on this link: https://usatinemedia.com/app/color-atlas-of-family-medicine/

The FP suspected acute systemic lupus erythematosus (SLE) with an acute cutaneous component. Laboratory testing showed a very high level of antinuclear antibodies. The patient was referred to Rheumatology and Dermatology. The dermatologist was available for a phone consult and suggested starting the patient on prednisone 60 mg/d as the Medrol Dosepak that the patient previously received had insufficient prednisolone for this severe flare of acute cutaneous lupus.

Although the classic description of acute SLE involves a butterfly rash, the rash of acute cutaneous lupus can include other areas of the face and body. As was seen in this case, the nasolabial fold tends to be spared and there are often skin erosions and crusting.

Based on the patient’s lab tests and symptoms, the dermatologist determined that the patient met the criteria for SLE. The patient was started on hydroxychloroquine 400 mg/d. The plan was to taper the patient’s prednisone slowly. By the following week, her skin and fatigue were much improved.

‌

Photos and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Pye A, Mayeaux EJ, Mishra V, et al. Lupus. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. Color Atlas and Synopsis of Family Medicine. 3rd ed. New York, NY: McGraw-Hill; 2019:1183-1193.

To learn more about the newest 3rd edition of the Color Atlas and Synopsis of Family Medicine, see: https://www.amazon.com/Color-Atlas-Synopsis-Family-Medicine/dp/1259862046/

You can get the 3rd edition of the Color Atlas and Synopsis of Family Medicine as an app by clicking on this link: https://usatinemedia.com/app/color-atlas-of-family-medicine/

The FP suspected acute systemic lupus erythematosus (SLE) with an acute cutaneous component. Laboratory testing showed a very high level of antinuclear antibodies. The patient was referred to Rheumatology and Dermatology. The dermatologist was available for a phone consult and suggested starting the patient on prednisone 60 mg/d as the Medrol Dosepak that the patient previously received had insufficient prednisolone for this severe flare of acute cutaneous lupus.

Although the classic description of acute SLE involves a butterfly rash, the rash of acute cutaneous lupus can include other areas of the face and body. As was seen in this case, the nasolabial fold tends to be spared and there are often skin erosions and crusting.

Based on the patient’s lab tests and symptoms, the dermatologist determined that the patient met the criteria for SLE. The patient was started on hydroxychloroquine 400 mg/d. The plan was to taper the patient’s prednisone slowly. By the following week, her skin and fatigue were much improved.

‌

Photos and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Pye A, Mayeaux EJ, Mishra V, et al. Lupus. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. Color Atlas and Synopsis of Family Medicine. 3rd ed. New York, NY: McGraw-Hill; 2019:1183-1193.

To learn more about the newest 3rd edition of the Color Atlas and Synopsis of Family Medicine, see: https://www.amazon.com/Color-Atlas-Synopsis-Family-Medicine/dp/1259862046/

You can get the 3rd edition of the Color Atlas and Synopsis of Family Medicine as an app by clicking on this link: https://usatinemedia.com/app/color-atlas-of-family-medicine/