Pigmentation Disorders

SAN DIEGO – According to J. Stuart Nelson, MD, PhD, three general principles guide the laser treatment of vascular skin lesions. The first is to target blood vessels beneath the surface of the skin.

“You’re going to be using wavelengths of light generally in the green and yellow portion of the spectrum,” Dr. Nelson, professor of surgery and biomedical engineering at the Beckman Laser Institute and Medical Clinic at the University of California, Irvine, said at the annual Masters of Aesthetics Symposium. “Blue light is highly absorbed by hemoglobin but unfortunately, blue light is highly scattered by human skin, so it won’t penetrate deep enough into the dermis. So primarily, we’re targeting hemoglobin using green and yellow light sources.”

The second principle is to match the pulse width with the vessel size, while the third is to give sufficient energy to irreversibly injure vessels based on selective photothermolysis.

Next, he advised clinicians to ask themselves three questions: What is the vessel size? “The larger the vessel, the longer the thermal relaxation time,” he said. What is the vessel depth? Deeper vessels require longer wavelengths of light and larger spot sizes. What is the patient’s skin phototype? Darker skin contains more epidermal melanin and requires extra caution during treatment.

Dr. Nelson listed seven optimal devices for the treatment of vascular skin lesions: intense pulsed light (IPL) with wavelengths of 515-1,200 nm and pulse durations of 1-10 ms, pulsed green light with a wavelength of 532 nm and pulse durations of 1-50 ms, pulsed dye yellow light with wavelengths of 585-600 nm and pulse durations of 0.5-40 ms, pulsed dye plus Nd:YAG with wavelengths of 595 and 1,064 nm and pulse durations of 0.5-40 ms, alexandrite laser with a wavelength of 755 nm and pulse durations of 0.25-100 ms, diode laser with a wavelength of 940 nm and pulse durations of 5-100 ms, and the pulsed Nd:YAG laser with a wavelength of 1,064 nm and pulse durations of 0.25-100 ms.

“You can get good results with every one of these devices,” Dr. Nelson said. “What you need to do is pick one and become what R. Rox Anderson, MD, calls an ‘endpointologist,’ so you can understand the clinical endpoints. Do not use a cookbook approach by trying to memorize treatment settings.”

Pulsed dye lasers with a wavelength of 585-600 nm have been the standard of care for years, he said, and is the treatment of choice for port wine stains in infants and young children. Upsides include the ability to treat large areas quickly and the ability to use two to three separate passes. It also induces diminution in diffuse redness and telangiectasia. Drawbacks include its potential to cause purpura when short pulse durations are used, it requires several treatments, it can be painful, and it causes considerable edema and erythema.

Millisecond green lasers at a wavelength of 532 nm are also effective for treating vascular skin lesions. “The nice thing about these devices is that you can focus them down to very small spots, so you can literally trace out individual blood vessels,” Dr. Nelson said. Other upsides include the fact that it can be performed without producing purpura, only transient erythema if few areas are treated. Drawbacks are that it’s moderately painful and may cause considerable edema. It also causes significant melanin absorption so is not advised for use in tanned and darker-skinned individuals. For all patients, contact cooling must be assured.

IPL, meanwhile, “can be very useful for treating not only vascular lesions, but also concurrently pigmented lesions such as poikiloderma of Civatte,” Dr. Nelson said. Potential drawbacks to IPL therapy are that the spectrum of light emitted and the pulse duration characteristics vary between devices and multiple treatments are required.

Finally, in the millisecond domain, the pulsed alexandrite 755-nm and Nd:YAG 1,064-nm lasers “are very good when trying to target something very deep in the skin like a vein,” he said. “But when you’re using those devices, you’re coagulating a large volume of tissue, so you need to be very careful about the amount of heat that you’re generating deep in the skin.”

When consulting with patients who have rosacea or telangiectasia, Dr. Nelson tells them multiple treatments will be required. “These are chronic conditions, and they may need ongoing maintenance treatments. The nice thing about all these procedures you’re doing for rosacea and telangiectasia is that they can be combined with all of your FDA [Food and Drug Administration]-approved topical and oral treatment protocols. All of these drugs you have at your disposal to medically treat rosacea can be all used concurrently with your laser treatment. When you see a patient you need to emphasize to them: ‘I’m not treating your rosacea with the laser. I’m treating a symptom of your rosacea with the laser.’ ”

Dr. Nelson closed his presentation by offering basic principles for success, the first being do no harm. “That’s the single most important thing you want to remember. No one will get mad if the blood vessel’s still there, but they’ll get very mad if something bad happens. You also want to underpromise and overdeliver. I always tell patients it’s going to require two to four treatments. When in doubt, don’t treat or undertreat. You can always treat again.”

If you’re concerned, perform a test spot. “There’s nothing wrong with that, particularly in a patient where you’re not sure what the outcome will be,” he said. “Check for any unusual skin reaction and for potential success of the procedure. Finally, don’t treat patients who are tanned.”

Dr. Nelson reported having intellectual property rights with Syneron Candela.

[email protected]

SAN DIEGO – According to J. Stuart Nelson, MD, PhD, three general principles guide the laser treatment of vascular skin lesions. The first is to target blood vessels beneath the surface of the skin.

“You’re going to be using wavelengths of light generally in the green and yellow portion of the spectrum,” Dr. Nelson, professor of surgery and biomedical engineering at the Beckman Laser Institute and Medical Clinic at the University of California, Irvine, said at the annual Masters of Aesthetics Symposium. “Blue light is highly absorbed by hemoglobin but unfortunately, blue light is highly scattered by human skin, so it won’t penetrate deep enough into the dermis. So primarily, we’re targeting hemoglobin using green and yellow light sources.”

The second principle is to match the pulse width with the vessel size, while the third is to give sufficient energy to irreversibly injure vessels based on selective photothermolysis.

Next, he advised clinicians to ask themselves three questions: What is the vessel size? “The larger the vessel, the longer the thermal relaxation time,” he said. What is the vessel depth? Deeper vessels require longer wavelengths of light and larger spot sizes. What is the patient’s skin phototype? Darker skin contains more epidermal melanin and requires extra caution during treatment.

Dr. Nelson listed seven optimal devices for the treatment of vascular skin lesions: intense pulsed light (IPL) with wavelengths of 515-1,200 nm and pulse durations of 1-10 ms, pulsed green light with a wavelength of 532 nm and pulse durations of 1-50 ms, pulsed dye yellow light with wavelengths of 585-600 nm and pulse durations of 0.5-40 ms, pulsed dye plus Nd:YAG with wavelengths of 595 and 1,064 nm and pulse durations of 0.5-40 ms, alexandrite laser with a wavelength of 755 nm and pulse durations of 0.25-100 ms, diode laser with a wavelength of 940 nm and pulse durations of 5-100 ms, and the pulsed Nd:YAG laser with a wavelength of 1,064 nm and pulse durations of 0.25-100 ms.

“You can get good results with every one of these devices,” Dr. Nelson said. “What you need to do is pick one and become what R. Rox Anderson, MD, calls an ‘endpointologist,’ so you can understand the clinical endpoints. Do not use a cookbook approach by trying to memorize treatment settings.”

Pulsed dye lasers with a wavelength of 585-600 nm have been the standard of care for years, he said, and is the treatment of choice for port wine stains in infants and young children. Upsides include the ability to treat large areas quickly and the ability to use two to three separate passes. It also induces diminution in diffuse redness and telangiectasia. Drawbacks include its potential to cause purpura when short pulse durations are used, it requires several treatments, it can be painful, and it causes considerable edema and erythema.

Millisecond green lasers at a wavelength of 532 nm are also effective for treating vascular skin lesions. “The nice thing about these devices is that you can focus them down to very small spots, so you can literally trace out individual blood vessels,” Dr. Nelson said. Other upsides include the fact that it can be performed without producing purpura, only transient erythema if few areas are treated. Drawbacks are that it’s moderately painful and may cause considerable edema. It also causes significant melanin absorption so is not advised for use in tanned and darker-skinned individuals. For all patients, contact cooling must be assured.

IPL, meanwhile, “can be very useful for treating not only vascular lesions, but also concurrently pigmented lesions such as poikiloderma of Civatte,” Dr. Nelson said. Potential drawbacks to IPL therapy are that the spectrum of light emitted and the pulse duration characteristics vary between devices and multiple treatments are required.

Finally, in the millisecond domain, the pulsed alexandrite 755-nm and Nd:YAG 1,064-nm lasers “are very good when trying to target something very deep in the skin like a vein,” he said. “But when you’re using those devices, you’re coagulating a large volume of tissue, so you need to be very careful about the amount of heat that you’re generating deep in the skin.”

When consulting with patients who have rosacea or telangiectasia, Dr. Nelson tells them multiple treatments will be required. “These are chronic conditions, and they may need ongoing maintenance treatments. The nice thing about all these procedures you’re doing for rosacea and telangiectasia is that they can be combined with all of your FDA [Food and Drug Administration]-approved topical and oral treatment protocols. All of these drugs you have at your disposal to medically treat rosacea can be all used concurrently with your laser treatment. When you see a patient you need to emphasize to them: ‘I’m not treating your rosacea with the laser. I’m treating a symptom of your rosacea with the laser.’ ”

Dr. Nelson closed his presentation by offering basic principles for success, the first being do no harm. “That’s the single most important thing you want to remember. No one will get mad if the blood vessel’s still there, but they’ll get very mad if something bad happens. You also want to underpromise and overdeliver. I always tell patients it’s going to require two to four treatments. When in doubt, don’t treat or undertreat. You can always treat again.”

If you’re concerned, perform a test spot. “There’s nothing wrong with that, particularly in a patient where you’re not sure what the outcome will be,” he said. “Check for any unusual skin reaction and for potential success of the procedure. Finally, don’t treat patients who are tanned.”

Dr. Nelson reported having intellectual property rights with Syneron Candela.

[email protected]

SAN DIEGO – According to J. Stuart Nelson, MD, PhD, three general principles guide the laser treatment of vascular skin lesions. The first is to target blood vessels beneath the surface of the skin.

“You’re going to be using wavelengths of light generally in the green and yellow portion of the spectrum,” Dr. Nelson, professor of surgery and biomedical engineering at the Beckman Laser Institute and Medical Clinic at the University of California, Irvine, said at the annual Masters of Aesthetics Symposium. “Blue light is highly absorbed by hemoglobin but unfortunately, blue light is highly scattered by human skin, so it won’t penetrate deep enough into the dermis. So primarily, we’re targeting hemoglobin using green and yellow light sources.”

The second principle is to match the pulse width with the vessel size, while the third is to give sufficient energy to irreversibly injure vessels based on selective photothermolysis.

Next, he advised clinicians to ask themselves three questions: What is the vessel size? “The larger the vessel, the longer the thermal relaxation time,” he said. What is the vessel depth? Deeper vessels require longer wavelengths of light and larger spot sizes. What is the patient’s skin phototype? Darker skin contains more epidermal melanin and requires extra caution during treatment.

Dr. Nelson listed seven optimal devices for the treatment of vascular skin lesions: intense pulsed light (IPL) with wavelengths of 515-1,200 nm and pulse durations of 1-10 ms, pulsed green light with a wavelength of 532 nm and pulse durations of 1-50 ms, pulsed dye yellow light with wavelengths of 585-600 nm and pulse durations of 0.5-40 ms, pulsed dye plus Nd:YAG with wavelengths of 595 and 1,064 nm and pulse durations of 0.5-40 ms, alexandrite laser with a wavelength of 755 nm and pulse durations of 0.25-100 ms, diode laser with a wavelength of 940 nm and pulse durations of 5-100 ms, and the pulsed Nd:YAG laser with a wavelength of 1,064 nm and pulse durations of 0.25-100 ms.

“You can get good results with every one of these devices,” Dr. Nelson said. “What you need to do is pick one and become what R. Rox Anderson, MD, calls an ‘endpointologist,’ so you can understand the clinical endpoints. Do not use a cookbook approach by trying to memorize treatment settings.”

Pulsed dye lasers with a wavelength of 585-600 nm have been the standard of care for years, he said, and is the treatment of choice for port wine stains in infants and young children. Upsides include the ability to treat large areas quickly and the ability to use two to three separate passes. It also induces diminution in diffuse redness and telangiectasia. Drawbacks include its potential to cause purpura when short pulse durations are used, it requires several treatments, it can be painful, and it causes considerable edema and erythema.

Millisecond green lasers at a wavelength of 532 nm are also effective for treating vascular skin lesions. “The nice thing about these devices is that you can focus them down to very small spots, so you can literally trace out individual blood vessels,” Dr. Nelson said. Other upsides include the fact that it can be performed without producing purpura, only transient erythema if few areas are treated. Drawbacks are that it’s moderately painful and may cause considerable edema. It also causes significant melanin absorption so is not advised for use in tanned and darker-skinned individuals. For all patients, contact cooling must be assured.

IPL, meanwhile, “can be very useful for treating not only vascular lesions, but also concurrently pigmented lesions such as poikiloderma of Civatte,” Dr. Nelson said. Potential drawbacks to IPL therapy are that the spectrum of light emitted and the pulse duration characteristics vary between devices and multiple treatments are required.

Finally, in the millisecond domain, the pulsed alexandrite 755-nm and Nd:YAG 1,064-nm lasers “are very good when trying to target something very deep in the skin like a vein,” he said. “But when you’re using those devices, you’re coagulating a large volume of tissue, so you need to be very careful about the amount of heat that you’re generating deep in the skin.”

When consulting with patients who have rosacea or telangiectasia, Dr. Nelson tells them multiple treatments will be required. “These are chronic conditions, and they may need ongoing maintenance treatments. The nice thing about all these procedures you’re doing for rosacea and telangiectasia is that they can be combined with all of your FDA [Food and Drug Administration]-approved topical and oral treatment protocols. All of these drugs you have at your disposal to medically treat rosacea can be all used concurrently with your laser treatment. When you see a patient you need to emphasize to them: ‘I’m not treating your rosacea with the laser. I’m treating a symptom of your rosacea with the laser.’ ”

Dr. Nelson closed his presentation by offering basic principles for success, the first being do no harm. “That’s the single most important thing you want to remember. No one will get mad if the blood vessel’s still there, but they’ll get very mad if something bad happens. You also want to underpromise and overdeliver. I always tell patients it’s going to require two to four treatments. When in doubt, don’t treat or undertreat. You can always treat again.”

If you’re concerned, perform a test spot. “There’s nothing wrong with that, particularly in a patient where you’re not sure what the outcome will be,” he said. “Check for any unusual skin reaction and for potential success of the procedure. Finally, don’t treat patients who are tanned.”

Dr. Nelson reported having intellectual property rights with Syneron Candela.

[email protected]

To the Editor:

A 17-year-old adolescent boy presented with dark spots on the legs and back of 2 months’ duration. He was not taking any medications and the spots could not be washed away by scrubbing with soap and water. He denied symptoms, except occasional itching. Family history revealed a maternal uncle with protein C deficiency and a maternal grandmother with systemic lupus erythematosus. Review of systems was negative; the patient denied joint pain and contact with heating pads or laptop computers. Based on the initial presentation, an underlying systemic condition was suspected. Physical examination revealed reticulate, nonblanching, brown patches on the bilateral arms, legs, and back in an apparent livedoid pattern (Figure). The patient’s history and physical examination suggested terra firma-forme dermatosis, livedo racemosa, or another vasculopathic process. However, gentle rubbing of the skin with an alcohol swab removed the discoloration completely, leading to the diagnosis of terra firma-forme dermatosis.

Livedo racemosa appears as an irregular, focal, reticulated discoloration of the skin.¹ The reticulated pattern of livedo racemosa has a branched or broken-up appearance.² Livedo racemosa indicates a disruption in the vasculature due to inflammation or occlusion.¹ The change is pathologic and does not blanch or resolve with warming.^1,2 The condition can progress to pigmentation and ulceration.¹ Livedo racemosa is a cutaneous manifestation of underlying vascular pathology. Due to a variety of causes, skin biopsy is nondiagnostic. Livedo racemosa can be caused by conditions such as systemic lupus erythematosus, syphilis, tuberculosis, polycythemia rubra vera, and Sneddon syndrome, among others.^3-5

Terra firma-forme dermatosis was reported in 1987 by Duncan et al.⁶ The condition classically presents with an exasperated mother who is unable to clean the “dirt” off her child’s skin despite multiple vigorous scrubbing attempts. The condition most commonly occurs in the summer months on the neck, face, and ankles.^7,8 Duncan et al⁶ reported that when the affected area was prepared for a biopsy, clean skin was revealed after wiping with an alcohol swab. No other cleansing agent has been reported to effectively remove the discoloration of terra firma-forme dermatosis. Hoping to elucidate a cause, Duncan et al⁶ performed both bacteriologic and fungal studies. The bacterial skin culture grew only normal flora, and fungal culture grew only normal contaminants consistent with the potassium hydroxide preparation of skin scraping. Histopathologic examination showed hyperkeratosis and orthokeratosis but not parakeratosis. Staining revealed melanin in the hyperkeratotic areas.⁶ Although the cause of this condition largely is unknown, it is thought that the epidermis in the affected areas could undergo altered maturation, resulting in trapping melanin that causes the skin to appear hyperkeratotic and hyperpigmented.¹ In our case, wiping the skin revealed the unsuspected diagnosis of terra firma-forme dermatosis displaying an unusual pseudolivedoid pattern. With apparently hyperpigmented processes, rubbing the skin with alcohol may help avoid unnecessary aggressive workup.

To the Editor:

A 17-year-old adolescent boy presented with dark spots on the legs and back of 2 months’ duration. He was not taking any medications and the spots could not be washed away by scrubbing with soap and water. He denied symptoms, except occasional itching. Family history revealed a maternal uncle with protein C deficiency and a maternal grandmother with systemic lupus erythematosus. Review of systems was negative; the patient denied joint pain and contact with heating pads or laptop computers. Based on the initial presentation, an underlying systemic condition was suspected. Physical examination revealed reticulate, nonblanching, brown patches on the bilateral arms, legs, and back in an apparent livedoid pattern (Figure). The patient’s history and physical examination suggested terra firma-forme dermatosis, livedo racemosa, or another vasculopathic process. However, gentle rubbing of the skin with an alcohol swab removed the discoloration completely, leading to the diagnosis of terra firma-forme dermatosis.

Livedo racemosa appears as an irregular, focal, reticulated discoloration of the skin.¹ The reticulated pattern of livedo racemosa has a branched or broken-up appearance.² Livedo racemosa indicates a disruption in the vasculature due to inflammation or occlusion.¹ The change is pathologic and does not blanch or resolve with warming.^1,2 The condition can progress to pigmentation and ulceration.¹ Livedo racemosa is a cutaneous manifestation of underlying vascular pathology. Due to a variety of causes, skin biopsy is nondiagnostic. Livedo racemosa can be caused by conditions such as systemic lupus erythematosus, syphilis, tuberculosis, polycythemia rubra vera, and Sneddon syndrome, among others.^3-5

Terra firma-forme dermatosis was reported in 1987 by Duncan et al.⁶ The condition classically presents with an exasperated mother who is unable to clean the “dirt” off her child’s skin despite multiple vigorous scrubbing attempts. The condition most commonly occurs in the summer months on the neck, face, and ankles.^7,8 Duncan et al⁶ reported that when the affected area was prepared for a biopsy, clean skin was revealed after wiping with an alcohol swab. No other cleansing agent has been reported to effectively remove the discoloration of terra firma-forme dermatosis. Hoping to elucidate a cause, Duncan et al⁶ performed both bacteriologic and fungal studies. The bacterial skin culture grew only normal flora, and fungal culture grew only normal contaminants consistent with the potassium hydroxide preparation of skin scraping. Histopathologic examination showed hyperkeratosis and orthokeratosis but not parakeratosis. Staining revealed melanin in the hyperkeratotic areas.⁶ Although the cause of this condition largely is unknown, it is thought that the epidermis in the affected areas could undergo altered maturation, resulting in trapping melanin that causes the skin to appear hyperkeratotic and hyperpigmented.¹ In our case, wiping the skin revealed the unsuspected diagnosis of terra firma-forme dermatosis displaying an unusual pseudolivedoid pattern. With apparently hyperpigmented processes, rubbing the skin with alcohol may help avoid unnecessary aggressive workup.

To the Editor:

A 17-year-old adolescent boy presented with dark spots on the legs and back of 2 months’ duration. He was not taking any medications and the spots could not be washed away by scrubbing with soap and water. He denied symptoms, except occasional itching. Family history revealed a maternal uncle with protein C deficiency and a maternal grandmother with systemic lupus erythematosus. Review of systems was negative; the patient denied joint pain and contact with heating pads or laptop computers. Based on the initial presentation, an underlying systemic condition was suspected. Physical examination revealed reticulate, nonblanching, brown patches on the bilateral arms, legs, and back in an apparent livedoid pattern (Figure). The patient’s history and physical examination suggested terra firma-forme dermatosis, livedo racemosa, or another vasculopathic process. However, gentle rubbing of the skin with an alcohol swab removed the discoloration completely, leading to the diagnosis of terra firma-forme dermatosis.

Livedo racemosa appears as an irregular, focal, reticulated discoloration of the skin.¹ The reticulated pattern of livedo racemosa has a branched or broken-up appearance.² Livedo racemosa indicates a disruption in the vasculature due to inflammation or occlusion.¹ The change is pathologic and does not blanch or resolve with warming.^1,2 The condition can progress to pigmentation and ulceration.¹ Livedo racemosa is a cutaneous manifestation of underlying vascular pathology. Due to a variety of causes, skin biopsy is nondiagnostic. Livedo racemosa can be caused by conditions such as systemic lupus erythematosus, syphilis, tuberculosis, polycythemia rubra vera, and Sneddon syndrome, among others.^3-5

Terra firma-forme dermatosis was reported in 1987 by Duncan et al.⁶ The condition classically presents with an exasperated mother who is unable to clean the “dirt” off her child’s skin despite multiple vigorous scrubbing attempts. The condition most commonly occurs in the summer months on the neck, face, and ankles.^7,8 Duncan et al⁶ reported that when the affected area was prepared for a biopsy, clean skin was revealed after wiping with an alcohol swab. No other cleansing agent has been reported to effectively remove the discoloration of terra firma-forme dermatosis. Hoping to elucidate a cause, Duncan et al⁶ performed both bacteriologic and fungal studies. The bacterial skin culture grew only normal flora, and fungal culture grew only normal contaminants consistent with the potassium hydroxide preparation of skin scraping. Histopathologic examination showed hyperkeratosis and orthokeratosis but not parakeratosis. Staining revealed melanin in the hyperkeratotic areas.⁶ Although the cause of this condition largely is unknown, it is thought that the epidermis in the affected areas could undergo altered maturation, resulting in trapping melanin that causes the skin to appear hyperkeratotic and hyperpigmented.¹ In our case, wiping the skin revealed the unsuspected diagnosis of terra firma-forme dermatosis displaying an unusual pseudolivedoid pattern. With apparently hyperpigmented processes, rubbing the skin with alcohol may help avoid unnecessary aggressive workup.

Extending oral propranolol treatment up to 12 months of age increased the success rate for high-risk infantile hemangioma, according to results published in Pediatrics.

Previous studies of oral propranol for infantile hemangiomas (IH) have revealed its efficacy, although there is no consensus on the optimal treatment duration. Nonetheless, treatment up to 12 months of age has been proposed if patients don’t respond after 6 months. Infants with high-risk hemangiomas, however, have been excluded from previous studies, authors of the current study explained.

In an open-label study of patients aged 35-150 days the success rate of oral propranolol was 47% after 6 months of treatment. The rate increased to 76% after the initial treatment period, reported Eulalia Baselga, MD, of the department of dermatology at Hospital de la Santa Creu i Sant Pau in Barcelona, and coauthors.

Investigators studied 45 patients from 10 hospitals in Spain and Poland between June 2015 and February 2017. The patients had high-risk IH in the proliferative phase. High-risk hemangiomas were defined as those that were life threatening, at risk for functional impact, disfiguring, or ulcerated nonresponsive to standard wound care measures.

Oral propranolol was administered twice daily at a dosage of 3 mg/kg per day. During the initial treatment period (ITP), patients received propranolol for a minimum of 6 months, and if treatment was not successful, it continued until success or up to 12 months of age.

Patients who achieved success in the initial phase were managed for 3 months with no treatment, and if there was rebound growth, treatment was restarted for up to 6 months at the provider’s discretion.

Treatment was considered a success if the target hemangioma resolved and there was no functional impact. The IH was considered resolved if it disappeared, even if there were minimal telangiectasias, erythema, skin thickening, soft tissue swelling, or the presence of sequelae.

Treatment success was achieved by 21 (47%) patients after 6 months and by 34 (76%) patients by the end of the ITP. Functional impact was determined using the Hemangioma Severity and Hemangioma Dynamic Complication scales. Adverse events, reported by 80% of patients, were resolved by the end of the study and included respiratory syncytial virus bronchiolitis, ulcerated hemangioma, pneumonia and respiratory failure, inguinal hernia, upper respiratory tract infection, dehydration, bronchitis, choking, and thermal burn. Although no patients experienced adverse events that resulted in discontinuation of treatment, 35 events led to temporary discontinuation, primarily due to respiratory events, the authors reported.

The results indicate that “oral propranolol is effective in treating high-risk IH with a favorable safety profile,” the authors concluded.

The study was funded by the Institut de Recherche Pierre Fabre Several authors were employed by or had other relationships with Pierre Fabre. The other authors had no conflicts of interest.

SOURCE: Baselga E et al. Pediatrics. 2018. doi: 10.1542/peds.2017-3866.

Extending oral propranolol treatment up to 12 months of age increased the success rate for high-risk infantile hemangioma, according to results published in Pediatrics.

Previous studies of oral propranol for infantile hemangiomas (IH) have revealed its efficacy, although there is no consensus on the optimal treatment duration. Nonetheless, treatment up to 12 months of age has been proposed if patients don’t respond after 6 months. Infants with high-risk hemangiomas, however, have been excluded from previous studies, authors of the current study explained.

In an open-label study of patients aged 35-150 days the success rate of oral propranolol was 47% after 6 months of treatment. The rate increased to 76% after the initial treatment period, reported Eulalia Baselga, MD, of the department of dermatology at Hospital de la Santa Creu i Sant Pau in Barcelona, and coauthors.

Investigators studied 45 patients from 10 hospitals in Spain and Poland between June 2015 and February 2017. The patients had high-risk IH in the proliferative phase. High-risk hemangiomas were defined as those that were life threatening, at risk for functional impact, disfiguring, or ulcerated nonresponsive to standard wound care measures.

Oral propranolol was administered twice daily at a dosage of 3 mg/kg per day. During the initial treatment period (ITP), patients received propranolol for a minimum of 6 months, and if treatment was not successful, it continued until success or up to 12 months of age.

Patients who achieved success in the initial phase were managed for 3 months with no treatment, and if there was rebound growth, treatment was restarted for up to 6 months at the provider’s discretion.

Treatment was considered a success if the target hemangioma resolved and there was no functional impact. The IH was considered resolved if it disappeared, even if there were minimal telangiectasias, erythema, skin thickening, soft tissue swelling, or the presence of sequelae.

Treatment success was achieved by 21 (47%) patients after 6 months and by 34 (76%) patients by the end of the ITP. Functional impact was determined using the Hemangioma Severity and Hemangioma Dynamic Complication scales. Adverse events, reported by 80% of patients, were resolved by the end of the study and included respiratory syncytial virus bronchiolitis, ulcerated hemangioma, pneumonia and respiratory failure, inguinal hernia, upper respiratory tract infection, dehydration, bronchitis, choking, and thermal burn. Although no patients experienced adverse events that resulted in discontinuation of treatment, 35 events led to temporary discontinuation, primarily due to respiratory events, the authors reported.

The results indicate that “oral propranolol is effective in treating high-risk IH with a favorable safety profile,” the authors concluded.

The study was funded by the Institut de Recherche Pierre Fabre Several authors were employed by or had other relationships with Pierre Fabre. The other authors had no conflicts of interest.

SOURCE: Baselga E et al. Pediatrics. 2018. doi: 10.1542/peds.2017-3866.

Extending oral propranolol treatment up to 12 months of age increased the success rate for high-risk infantile hemangioma, according to results published in Pediatrics.

Previous studies of oral propranol for infantile hemangiomas (IH) have revealed its efficacy, although there is no consensus on the optimal treatment duration. Nonetheless, treatment up to 12 months of age has been proposed if patients don’t respond after 6 months. Infants with high-risk hemangiomas, however, have been excluded from previous studies, authors of the current study explained.

In an open-label study of patients aged 35-150 days the success rate of oral propranolol was 47% after 6 months of treatment. The rate increased to 76% after the initial treatment period, reported Eulalia Baselga, MD, of the department of dermatology at Hospital de la Santa Creu i Sant Pau in Barcelona, and coauthors.

Investigators studied 45 patients from 10 hospitals in Spain and Poland between June 2015 and February 2017. The patients had high-risk IH in the proliferative phase. High-risk hemangiomas were defined as those that were life threatening, at risk for functional impact, disfiguring, or ulcerated nonresponsive to standard wound care measures.

Oral propranolol was administered twice daily at a dosage of 3 mg/kg per day. During the initial treatment period (ITP), patients received propranolol for a minimum of 6 months, and if treatment was not successful, it continued until success or up to 12 months of age.

Patients who achieved success in the initial phase were managed for 3 months with no treatment, and if there was rebound growth, treatment was restarted for up to 6 months at the provider’s discretion.

Treatment was considered a success if the target hemangioma resolved and there was no functional impact. The IH was considered resolved if it disappeared, even if there were minimal telangiectasias, erythema, skin thickening, soft tissue swelling, or the presence of sequelae.

Treatment success was achieved by 21 (47%) patients after 6 months and by 34 (76%) patients by the end of the ITP. Functional impact was determined using the Hemangioma Severity and Hemangioma Dynamic Complication scales. Adverse events, reported by 80% of patients, were resolved by the end of the study and included respiratory syncytial virus bronchiolitis, ulcerated hemangioma, pneumonia and respiratory failure, inguinal hernia, upper respiratory tract infection, dehydration, bronchitis, choking, and thermal burn. Although no patients experienced adverse events that resulted in discontinuation of treatment, 35 events led to temporary discontinuation, primarily due to respiratory events, the authors reported.

The results indicate that “oral propranolol is effective in treating high-risk IH with a favorable safety profile,” the authors concluded.

The study was funded by the Institut de Recherche Pierre Fabre Several authors were employed by or had other relationships with Pierre Fabre. The other authors had no conflicts of interest.

SOURCE: Baselga E et al. Pediatrics. 2018. doi: 10.1542/peds.2017-3866.

LAKE TAHOE, CALIF. – Several years ago, David H. Darrow, MD, DDS, began to notice a pattern in the conversation threads on websites dedicated to support for parents of children with facial port-wine stains (PWS).

Parents were reporting that dental problems arose earlier on their child’s side of face with the PWS, and that the alveolar ridge was lower on the side of the face that harbored the lesion. “Most importantly, parents were concerned that dentists were not touching their children because they were concerned about bleeding,” Dr. Darrow said at the annual meeting of the Society for Pediatric Dermatology. A search in the medical literature for port-wine stains and oral cavity changes, did not turn up much except for a few articles on bleeding. “One said that port-wine stains or capillary malformations rarely present major problems for the oral and maxillofacial surgeon. The other said that periodontal probing should not be done, as even gentle probing can result in uncontrolled bleeding,” he noted.

This prompted Dr. Darrow, who directs the Center for Hemangiomas and Vascular Birthmarks at the Eastern Virginia Medical School, Norfolk, and coinvestigators, Megan B. Dowling, MD, and Yueqin Zhao, PhD, to characterize manifestations of PWS in the oral cavity via an anonymous paired survey of volunteers with facial PWS and their dentists who were recruited from birthmarks.com and 10 collaborating physician practices (J Am Acad Dermatol 2012;67:687-93). Volunteers ranged in age from 1 to 62 years; mean age was 29 years. A total of 30 patients participated, and most (67%) were female.

The five most common oral manifestations reported by the patients were lip hyperplasia (53%), stained gingiva (47%), malocclusion (30%), bleeding gingiva (27%), and spacing between teeth (23%). Only 3% reported bleeding from dental procedures. When the researchers evaluated the orodental findings in the paired patient-physician responses, “most of the time there was good agreement between the patient and the dentist,” Dr. Darrow said. “The only one that fell out of agreement was lip hyperplasia. That’s probably because most dentists look right past the lips and into the oral cavity.”

When the researchers examined patients who had stained gingiva versus those who did not, they found that early-stage lesions demonstrated a reddish blush of the oral mucosa and gingiva, while late-stage lesions demonstrated increased blush of the oral tissues, as well as hyperplasia of the soft tissue or bone in the affected area. “Based on our review of the literature, bleeding of gums is rarely prolonged and dental procedures are safe,” Dr. Darrow said.

The findings are important, he continued, because capillary malformations of the oral cavity may result in periodontal disease associated with gingival overgrowth. The depth of the gingival pocket should be no more than 2-3 mm. “When you have areas of inflammation and deep-pocket formation, plaque and bacteria slowly erode the connection between the tooth and the soft tissue,” he explained. “At some point, that pocket becomes so deep that it reaches down into the bone in which the tooth is anchored. As that bone is eroded, the teeth loosen and begin to fall out. The goals of therapy are prevention of periodontal disease with meticulous oral hygiene.”

Soft tissue hyperplasia may be exacerbated by medications such as calcium channel blockers, cyclosporine, and phenytoin and phenobarbital, which are sometimes used by patients with Sturge-Weber syndrome, he said.

Dr. Darrow reported having no financial disclosures.

[email protected]

LAKE TAHOE, CALIF. – Several years ago, David H. Darrow, MD, DDS, began to notice a pattern in the conversation threads on websites dedicated to support for parents of children with facial port-wine stains (PWS).

Parents were reporting that dental problems arose earlier on their child’s side of face with the PWS, and that the alveolar ridge was lower on the side of the face that harbored the lesion. “Most importantly, parents were concerned that dentists were not touching their children because they were concerned about bleeding,” Dr. Darrow said at the annual meeting of the Society for Pediatric Dermatology. A search in the medical literature for port-wine stains and oral cavity changes, did not turn up much except for a few articles on bleeding. “One said that port-wine stains or capillary malformations rarely present major problems for the oral and maxillofacial surgeon. The other said that periodontal probing should not be done, as even gentle probing can result in uncontrolled bleeding,” he noted.

This prompted Dr. Darrow, who directs the Center for Hemangiomas and Vascular Birthmarks at the Eastern Virginia Medical School, Norfolk, and coinvestigators, Megan B. Dowling, MD, and Yueqin Zhao, PhD, to characterize manifestations of PWS in the oral cavity via an anonymous paired survey of volunteers with facial PWS and their dentists who were recruited from birthmarks.com and 10 collaborating physician practices (J Am Acad Dermatol 2012;67:687-93). Volunteers ranged in age from 1 to 62 years; mean age was 29 years. A total of 30 patients participated, and most (67%) were female.

The five most common oral manifestations reported by the patients were lip hyperplasia (53%), stained gingiva (47%), malocclusion (30%), bleeding gingiva (27%), and spacing between teeth (23%). Only 3% reported bleeding from dental procedures. When the researchers evaluated the orodental findings in the paired patient-physician responses, “most of the time there was good agreement between the patient and the dentist,” Dr. Darrow said. “The only one that fell out of agreement was lip hyperplasia. That’s probably because most dentists look right past the lips and into the oral cavity.”

When the researchers examined patients who had stained gingiva versus those who did not, they found that early-stage lesions demonstrated a reddish blush of the oral mucosa and gingiva, while late-stage lesions demonstrated increased blush of the oral tissues, as well as hyperplasia of the soft tissue or bone in the affected area. “Based on our review of the literature, bleeding of gums is rarely prolonged and dental procedures are safe,” Dr. Darrow said.

The findings are important, he continued, because capillary malformations of the oral cavity may result in periodontal disease associated with gingival overgrowth. The depth of the gingival pocket should be no more than 2-3 mm. “When you have areas of inflammation and deep-pocket formation, plaque and bacteria slowly erode the connection between the tooth and the soft tissue,” he explained. “At some point, that pocket becomes so deep that it reaches down into the bone in which the tooth is anchored. As that bone is eroded, the teeth loosen and begin to fall out. The goals of therapy are prevention of periodontal disease with meticulous oral hygiene.”

Soft tissue hyperplasia may be exacerbated by medications such as calcium channel blockers, cyclosporine, and phenytoin and phenobarbital, which are sometimes used by patients with Sturge-Weber syndrome, he said.

Dr. Darrow reported having no financial disclosures.

[email protected]

LAKE TAHOE, CALIF. – Several years ago, David H. Darrow, MD, DDS, began to notice a pattern in the conversation threads on websites dedicated to support for parents of children with facial port-wine stains (PWS).

Parents were reporting that dental problems arose earlier on their child’s side of face with the PWS, and that the alveolar ridge was lower on the side of the face that harbored the lesion. “Most importantly, parents were concerned that dentists were not touching their children because they were concerned about bleeding,” Dr. Darrow said at the annual meeting of the Society for Pediatric Dermatology. A search in the medical literature for port-wine stains and oral cavity changes, did not turn up much except for a few articles on bleeding. “One said that port-wine stains or capillary malformations rarely present major problems for the oral and maxillofacial surgeon. The other said that periodontal probing should not be done, as even gentle probing can result in uncontrolled bleeding,” he noted.

This prompted Dr. Darrow, who directs the Center for Hemangiomas and Vascular Birthmarks at the Eastern Virginia Medical School, Norfolk, and coinvestigators, Megan B. Dowling, MD, and Yueqin Zhao, PhD, to characterize manifestations of PWS in the oral cavity via an anonymous paired survey of volunteers with facial PWS and their dentists who were recruited from birthmarks.com and 10 collaborating physician practices (J Am Acad Dermatol 2012;67:687-93). Volunteers ranged in age from 1 to 62 years; mean age was 29 years. A total of 30 patients participated, and most (67%) were female.

The five most common oral manifestations reported by the patients were lip hyperplasia (53%), stained gingiva (47%), malocclusion (30%), bleeding gingiva (27%), and spacing between teeth (23%). Only 3% reported bleeding from dental procedures. When the researchers evaluated the orodental findings in the paired patient-physician responses, “most of the time there was good agreement between the patient and the dentist,” Dr. Darrow said. “The only one that fell out of agreement was lip hyperplasia. That’s probably because most dentists look right past the lips and into the oral cavity.”

When the researchers examined patients who had stained gingiva versus those who did not, they found that early-stage lesions demonstrated a reddish blush of the oral mucosa and gingiva, while late-stage lesions demonstrated increased blush of the oral tissues, as well as hyperplasia of the soft tissue or bone in the affected area. “Based on our review of the literature, bleeding of gums is rarely prolonged and dental procedures are safe,” Dr. Darrow said.

The findings are important, he continued, because capillary malformations of the oral cavity may result in periodontal disease associated with gingival overgrowth. The depth of the gingival pocket should be no more than 2-3 mm. “When you have areas of inflammation and deep-pocket formation, plaque and bacteria slowly erode the connection between the tooth and the soft tissue,” he explained. “At some point, that pocket becomes so deep that it reaches down into the bone in which the tooth is anchored. As that bone is eroded, the teeth loosen and begin to fall out. The goals of therapy are prevention of periodontal disease with meticulous oral hygiene.”

Soft tissue hyperplasia may be exacerbated by medications such as calcium channel blockers, cyclosporine, and phenytoin and phenobarbital, which are sometimes used by patients with Sturge-Weber syndrome, he said.

Dr. Darrow reported having no financial disclosures.

[email protected]

To the Editor:

Morphea, atrophoderma, guttate lichen sclerosus et atrophicus (LS&A), anetoderma, and their subtypes are inflammatory processes ultimately leading to dermal remodeling. We report a case of a scaly, hypopigmented, macular rash that clinically appeared as an entity along the morphea-atrophoderma spectrum and demonstrated unique histopathologic changes in both collagen and elastin confined to the upper reticular and papillary dermis. This case is a potentially rare variant representing a combination of clinical and microscopic findings.

A 29-year-old woman presented for an increasing number of white spots distributed on the trunk, arms, and legs. She denied local and systemic symptoms. The patient reported that she was stung by 100 wasps 23 years prior. Following the assault, her grandmother placed chewed tobacco leaves atop the painful erythematous wheals and flares. Upon resolution, hypopigmented macules and patches remained in their place. The patient denied associated symptoms or new lesions; she did not seek care at that time.

In her early 20s, the patient noted new, similarly distributed hypopigmented macules and patches without associated arthropod assault. She was treated by an outside dermatologist without result for presumed tinea versicolor. A follow-up superficial shave biopsy cited subtle psoriasiform dermatitis. Topical steroids did not improve the lesions. Her medical history also was remarkable for a reportedly unprovoked complete rotator cuff tear.

Physical examination revealed 0.5- to 2.0-cm, ill-defined, perifollicular and nonfollicular, slightly scaly macules and patches on the trunk, arms, and legs. There was no follicular plugging (Figure 1A). The hands, feet, face, and mucosal surfaces were spared. She had no family history of similar lesions. Although atrophic in appearance, a single lesion on the left thigh was palpably depressed (Figure 1B). Serology demonstrated a normal complete blood cell count and comprehensive metabolic panel, and negative Lyme titers. Light therapy and topical steroids failed to improve the lesions; calcipotriene cream 0.005% made the lesions erythematous and pruritic.

A biopsy from a flank lesion demonstrated a normal epithelium without thinning, a normal basal melanocyte population, and minimally effaced rete ridges. Thin collagen bundles were noted in the upper reticular and papillary dermis with associated fibroplasia (Figure 2). Verhoeff-van Gieson stain revealed decreased and fragmented elastin filaments in the same dermal distribution as the changed collagen (Figure 3). There was no evidence of primary inflammatory disease. The dermis was thinned. Periodic acid–Schiff stain confirmed the absence of hyphae and spores.

The relevant findings in our patient including the following: (1) onset of hypopigmented macules and patches following resolution of a toxic insult; (2) initially stable number of lesions that progressed in number but not size; (3) thinned collagen associated with fibroplasia in the upper reticular and papillary dermis; (4) decreased number and fragmentation of elastin filaments confined to the same region; (5) no congenital lesions or similar lesions in family members; and (6) a complete rotator cuff tear with no findings of a systemic connective-tissue disorder such as Ehlers-Danlos syndrome.

We performed a literature search of PubMed articles indexed for MEDLINE using combinations of the terms atrophic, hypopigmented, white, spot disease, confetti-like, guttate, macules, atrophoderma, morphea, anetoderma, elastin, and collagen to identify potentially similar reports of guttate hypopigmented macules demonstrating changes of the collagen and elastin in the papillary and upper reticular dermis. Some variants, namely atrophoderma of Pasini and Pierini (APP), guttate morphea, and superficial morphea, demonstrate similar clinical and histopathologic findings.

Findings similar to our case were documented in case reports of 2 women (aged 34 and 42 years)¹ presenting with asymptomatic, atrophic, well-demarcated, shiny, hypopigmented macules over the trunk and upper extremities, which demonstrated a thinned epidermis with coarse hyalinized collagen bundles in the mid and lower dermis. There was upper and diffuse dermal elastolysis (patient 1 and patient 2, respectively).¹ Our patient’s lesions were hypopigmented and atrophic in appearance but were slightly scaly and also involved the extremities. Distinct from these patient reports, histopathology from our case demonstrated thin packed collagen bundles and decreased fragmented elastin filaments confined to the upper reticular and papillary dermis.

Plaque morphea is the most common type of localized scleroderma.² The subtype APP demonstrates round to ovoid, gray-brown depressions with cliff-drop borders. They may appear flesh colored or hypopigmented.^3,4 These sclerodermoid lesions lack the violaceous border classic to morphea. Sclerosis and induration also are typically absent.⁵ Clinically, our patient’s macules resembled this entity. Histopathologically, APP shows normal epithelium with an increased basal layer pigmentation; preserved adnexal structures; and mid to lower dermal collagen edema, clumping, and homogenization.^3,4 Elastic fibers classically are unchanged, with exceptions.^6-11 Changes in the collagen and elastin of our patient were unlike those reported in APP, which occur in the mid to lower dermis.

Guttate morphea demonstrates small, pale, minimally indurated, coin-shaped lesions on the trunk. Histopathology reveals less sclerosis and more edema, resembling LS&A.¹² The earliest descriptions of this entity describe 3 stages: ivory/chalk white, scaly, and atrophic. Follicular plugging (absent in this patient) and fine scale can exist at any stage.^13,14 Flattened rete ridges mark an otherwise preserved epidermis; hyalinized collagen typically is superficial and demonstrates less sclerosis yet increased edema.^12-14 Fewer elastic fibers typically are present compared to normal skin. Changes seen in this entity are more superficial, as with our patient, than classic scleroderma. However, classic edema was not found in our patient’s biopsy specimen.

Superficial morphea, occurring predominantly in females, presents with hyperpigmented or hypopigmented patches having minimal to no induration. The lesions typically are asymptomatic. Histopathologically, collagen deposition and inflammation are confined to the superficial dermis without homogenization associated with LS&A, findings that were consistent with this patient’s biopsy.^15,16 However, similar to other morpheaform variants, elastic fibers are unchanged.¹⁵ Verhoeff-van Gieson stain of the biopsy (Figure 3) showed the decreased and fragmented elastin network in the upper reticular and papillary dermis, making this entity less compatible.

Guttate LS&A may present with interfollicular, bluish white macules or papules coalescing into patches or plaques. Lesions evolve to reveal atrophic thin skin with follicular plugging. Histology demonstrates a thinned epidermis with orthohypokeratosis marked by flattened rete ridges. The dermis reveals short hyalinized collagen fibrils with a loss of elastic fibers in the papillary and upper reticular dermis, giving a homogenized appearance. Early disease is marked by an inflammatory infiltrate.¹⁷ Most of these findings are consistent with our patient’s pathology, which was confined to the upper dermis. Lacking, however, were characteristic findings of LS&A, including upper dermal homogenization, near-total effacement of rete ridges, orthokeratosis, and vacuolar degeneration at the dermoepidermal junction. As such, this entity is less compatible.

Atrophoderma elastolyticum discretum has clinical features of atrophoderma with elastolytic histopathologic findings.¹ Anetoderma presents with outpouchings of atrophic skin with a surrounding ring of normal tissue. Histopathologically, this entity shows normal collagen with elastolysis; there also is a decrease in desmosine, an elastin cross-linker.^1,3 Neither the clinical nor histopathologic findings in this patient matched these 2 entities.

The reported chronologic association of these lesions with an arthropod assault raised suspicion to their association with toxic insult or postinflammatory changes. One study reported mechanical trauma, including insect bites, as a possible inciting factor of morphea.¹¹ These data, gathered from patient surveys, reported trauma associated to lesion development.^1,17 A review of the literature regarding atrophoderma, morphea, and LS&A failed to identify pathogenic changes seen in this patient following initial trauma. Moreover, although it is difficult to prove causality in the formation of the original hypopigmented spots, the development of identical spots in a similar distribution without further trauma suggests against these etiologies to fully explain her lesions. Nonetheless, circumstance makes it difficult to prove whether the original arthropod insult spurred a smoldering reactive process that caused the newer lesions.

Hereditary connective-tissue disorders also were considered in the differential diagnosis. Because of the patient’s history of an unprovoked complete rotator cuff tear, Ehlers-Danlos syndrome was considered; however, the remainder of her examination was normal, making a syndromic systemic disorder a less likely etiology.Because of the distinct clinical and histopathologic findings, this case may represent a rare and previously unreported variant of morphea. Clinically, these hypopigmented macules and patches exist somewhere along the morphea-atrophoderma spectrum. Histopathologic findings do not conform to prior reports. The name atrophodermalike guttate morphea may be an appropriate appellation. It is possible this presentation represents a variant of what dermatologists have referred to as white spot disease.¹⁸ We hope that this case may bring others to discussion, allowing for the identification of a more precise entity and etiology so that patients may receive more directed therapy.

To the Editor:

Morphea, atrophoderma, guttate lichen sclerosus et atrophicus (LS&A), anetoderma, and their subtypes are inflammatory processes ultimately leading to dermal remodeling. We report a case of a scaly, hypopigmented, macular rash that clinically appeared as an entity along the morphea-atrophoderma spectrum and demonstrated unique histopathologic changes in both collagen and elastin confined to the upper reticular and papillary dermis. This case is a potentially rare variant representing a combination of clinical and microscopic findings.

A 29-year-old woman presented for an increasing number of white spots distributed on the trunk, arms, and legs. She denied local and systemic symptoms. The patient reported that she was stung by 100 wasps 23 years prior. Following the assault, her grandmother placed chewed tobacco leaves atop the painful erythematous wheals and flares. Upon resolution, hypopigmented macules and patches remained in their place. The patient denied associated symptoms or new lesions; she did not seek care at that time.

In her early 20s, the patient noted new, similarly distributed hypopigmented macules and patches without associated arthropod assault. She was treated by an outside dermatologist without result for presumed tinea versicolor. A follow-up superficial shave biopsy cited subtle psoriasiform dermatitis. Topical steroids did not improve the lesions. Her medical history also was remarkable for a reportedly unprovoked complete rotator cuff tear.

Physical examination revealed 0.5- to 2.0-cm, ill-defined, perifollicular and nonfollicular, slightly scaly macules and patches on the trunk, arms, and legs. There was no follicular plugging (Figure 1A). The hands, feet, face, and mucosal surfaces were spared. She had no family history of similar lesions. Although atrophic in appearance, a single lesion on the left thigh was palpably depressed (Figure 1B). Serology demonstrated a normal complete blood cell count and comprehensive metabolic panel, and negative Lyme titers. Light therapy and topical steroids failed to improve the lesions; calcipotriene cream 0.005% made the lesions erythematous and pruritic.

A biopsy from a flank lesion demonstrated a normal epithelium without thinning, a normal basal melanocyte population, and minimally effaced rete ridges. Thin collagen bundles were noted in the upper reticular and papillary dermis with associated fibroplasia (Figure 2). Verhoeff-van Gieson stain revealed decreased and fragmented elastin filaments in the same dermal distribution as the changed collagen (Figure 3). There was no evidence of primary inflammatory disease. The dermis was thinned. Periodic acid–Schiff stain confirmed the absence of hyphae and spores.

The relevant findings in our patient including the following: (1) onset of hypopigmented macules and patches following resolution of a toxic insult; (2) initially stable number of lesions that progressed in number but not size; (3) thinned collagen associated with fibroplasia in the upper reticular and papillary dermis; (4) decreased number and fragmentation of elastin filaments confined to the same region; (5) no congenital lesions or similar lesions in family members; and (6) a complete rotator cuff tear with no findings of a systemic connective-tissue disorder such as Ehlers-Danlos syndrome.

We performed a literature search of PubMed articles indexed for MEDLINE using combinations of the terms atrophic, hypopigmented, white, spot disease, confetti-like, guttate, macules, atrophoderma, morphea, anetoderma, elastin, and collagen to identify potentially similar reports of guttate hypopigmented macules demonstrating changes of the collagen and elastin in the papillary and upper reticular dermis. Some variants, namely atrophoderma of Pasini and Pierini (APP), guttate morphea, and superficial morphea, demonstrate similar clinical and histopathologic findings.

Findings similar to our case were documented in case reports of 2 women (aged 34 and 42 years)¹ presenting with asymptomatic, atrophic, well-demarcated, shiny, hypopigmented macules over the trunk and upper extremities, which demonstrated a thinned epidermis with coarse hyalinized collagen bundles in the mid and lower dermis. There was upper and diffuse dermal elastolysis (patient 1 and patient 2, respectively).¹ Our patient’s lesions were hypopigmented and atrophic in appearance but were slightly scaly and also involved the extremities. Distinct from these patient reports, histopathology from our case demonstrated thin packed collagen bundles and decreased fragmented elastin filaments confined to the upper reticular and papillary dermis.

Plaque morphea is the most common type of localized scleroderma.² The subtype APP demonstrates round to ovoid, gray-brown depressions with cliff-drop borders. They may appear flesh colored or hypopigmented.^3,4 These sclerodermoid lesions lack the violaceous border classic to morphea. Sclerosis and induration also are typically absent.⁵ Clinically, our patient’s macules resembled this entity. Histopathologically, APP shows normal epithelium with an increased basal layer pigmentation; preserved adnexal structures; and mid to lower dermal collagen edema, clumping, and homogenization.^3,4 Elastic fibers classically are unchanged, with exceptions.^6-11 Changes in the collagen and elastin of our patient were unlike those reported in APP, which occur in the mid to lower dermis.

Guttate morphea demonstrates small, pale, minimally indurated, coin-shaped lesions on the trunk. Histopathology reveals less sclerosis and more edema, resembling LS&A.¹² The earliest descriptions of this entity describe 3 stages: ivory/chalk white, scaly, and atrophic. Follicular plugging (absent in this patient) and fine scale can exist at any stage.^13,14 Flattened rete ridges mark an otherwise preserved epidermis; hyalinized collagen typically is superficial and demonstrates less sclerosis yet increased edema.^12-14 Fewer elastic fibers typically are present compared to normal skin. Changes seen in this entity are more superficial, as with our patient, than classic scleroderma. However, classic edema was not found in our patient’s biopsy specimen.

Superficial morphea, occurring predominantly in females, presents with hyperpigmented or hypopigmented patches having minimal to no induration. The lesions typically are asymptomatic. Histopathologically, collagen deposition and inflammation are confined to the superficial dermis without homogenization associated with LS&A, findings that were consistent with this patient’s biopsy.^15,16 However, similar to other morpheaform variants, elastic fibers are unchanged.¹⁵ Verhoeff-van Gieson stain of the biopsy (Figure 3) showed the decreased and fragmented elastin network in the upper reticular and papillary dermis, making this entity less compatible.

Guttate LS&A may present with interfollicular, bluish white macules or papules coalescing into patches or plaques. Lesions evolve to reveal atrophic thin skin with follicular plugging. Histology demonstrates a thinned epidermis with orthohypokeratosis marked by flattened rete ridges. The dermis reveals short hyalinized collagen fibrils with a loss of elastic fibers in the papillary and upper reticular dermis, giving a homogenized appearance. Early disease is marked by an inflammatory infiltrate.¹⁷ Most of these findings are consistent with our patient’s pathology, which was confined to the upper dermis. Lacking, however, were characteristic findings of LS&A, including upper dermal homogenization, near-total effacement of rete ridges, orthokeratosis, and vacuolar degeneration at the dermoepidermal junction. As such, this entity is less compatible.

Atrophoderma elastolyticum discretum has clinical features of atrophoderma with elastolytic histopathologic findings.¹ Anetoderma presents with outpouchings of atrophic skin with a surrounding ring of normal tissue. Histopathologically, this entity shows normal collagen with elastolysis; there also is a decrease in desmosine, an elastin cross-linker.^1,3 Neither the clinical nor histopathologic findings in this patient matched these 2 entities.

The reported chronologic association of these lesions with an arthropod assault raised suspicion to their association with toxic insult or postinflammatory changes. One study reported mechanical trauma, including insect bites, as a possible inciting factor of morphea.¹¹ These data, gathered from patient surveys, reported trauma associated to lesion development.^1,17 A review of the literature regarding atrophoderma, morphea, and LS&A failed to identify pathogenic changes seen in this patient following initial trauma. Moreover, although it is difficult to prove causality in the formation of the original hypopigmented spots, the development of identical spots in a similar distribution without further trauma suggests against these etiologies to fully explain her lesions. Nonetheless, circumstance makes it difficult to prove whether the original arthropod insult spurred a smoldering reactive process that caused the newer lesions.

Hereditary connective-tissue disorders also were considered in the differential diagnosis. Because of the patient’s history of an unprovoked complete rotator cuff tear, Ehlers-Danlos syndrome was considered; however, the remainder of her examination was normal, making a syndromic systemic disorder a less likely etiology.Because of the distinct clinical and histopathologic findings, this case may represent a rare and previously unreported variant of morphea. Clinically, these hypopigmented macules and patches exist somewhere along the morphea-atrophoderma spectrum. Histopathologic findings do not conform to prior reports. The name atrophodermalike guttate morphea may be an appropriate appellation. It is possible this presentation represents a variant of what dermatologists have referred to as white spot disease.¹⁸ We hope that this case may bring others to discussion, allowing for the identification of a more precise entity and etiology so that patients may receive more directed therapy.

To the Editor:

Morphea, atrophoderma, guttate lichen sclerosus et atrophicus (LS&A), anetoderma, and their subtypes are inflammatory processes ultimately leading to dermal remodeling. We report a case of a scaly, hypopigmented, macular rash that clinically appeared as an entity along the morphea-atrophoderma spectrum and demonstrated unique histopathologic changes in both collagen and elastin confined to the upper reticular and papillary dermis. This case is a potentially rare variant representing a combination of clinical and microscopic findings.

A 29-year-old woman presented for an increasing number of white spots distributed on the trunk, arms, and legs. She denied local and systemic symptoms. The patient reported that she was stung by 100 wasps 23 years prior. Following the assault, her grandmother placed chewed tobacco leaves atop the painful erythematous wheals and flares. Upon resolution, hypopigmented macules and patches remained in their place. The patient denied associated symptoms or new lesions; she did not seek care at that time.

In her early 20s, the patient noted new, similarly distributed hypopigmented macules and patches without associated arthropod assault. She was treated by an outside dermatologist without result for presumed tinea versicolor. A follow-up superficial shave biopsy cited subtle psoriasiform dermatitis. Topical steroids did not improve the lesions. Her medical history also was remarkable for a reportedly unprovoked complete rotator cuff tear.

Physical examination revealed 0.5- to 2.0-cm, ill-defined, perifollicular and nonfollicular, slightly scaly macules and patches on the trunk, arms, and legs. There was no follicular plugging (Figure 1A). The hands, feet, face, and mucosal surfaces were spared. She had no family history of similar lesions. Although atrophic in appearance, a single lesion on the left thigh was palpably depressed (Figure 1B). Serology demonstrated a normal complete blood cell count and comprehensive metabolic panel, and negative Lyme titers. Light therapy and topical steroids failed to improve the lesions; calcipotriene cream 0.005% made the lesions erythematous and pruritic.

A biopsy from a flank lesion demonstrated a normal epithelium without thinning, a normal basal melanocyte population, and minimally effaced rete ridges. Thin collagen bundles were noted in the upper reticular and papillary dermis with associated fibroplasia (Figure 2). Verhoeff-van Gieson stain revealed decreased and fragmented elastin filaments in the same dermal distribution as the changed collagen (Figure 3). There was no evidence of primary inflammatory disease. The dermis was thinned. Periodic acid–Schiff stain confirmed the absence of hyphae and spores.

The relevant findings in our patient including the following: (1) onset of hypopigmented macules and patches following resolution of a toxic insult; (2) initially stable number of lesions that progressed in number but not size; (3) thinned collagen associated with fibroplasia in the upper reticular and papillary dermis; (4) decreased number and fragmentation of elastin filaments confined to the same region; (5) no congenital lesions or similar lesions in family members; and (6) a complete rotator cuff tear with no findings of a systemic connective-tissue disorder such as Ehlers-Danlos syndrome.

We performed a literature search of PubMed articles indexed for MEDLINE using combinations of the terms atrophic, hypopigmented, white, spot disease, confetti-like, guttate, macules, atrophoderma, morphea, anetoderma, elastin, and collagen to identify potentially similar reports of guttate hypopigmented macules demonstrating changes of the collagen and elastin in the papillary and upper reticular dermis. Some variants, namely atrophoderma of Pasini and Pierini (APP), guttate morphea, and superficial morphea, demonstrate similar clinical and histopathologic findings.

Findings similar to our case were documented in case reports of 2 women (aged 34 and 42 years)¹ presenting with asymptomatic, atrophic, well-demarcated, shiny, hypopigmented macules over the trunk and upper extremities, which demonstrated a thinned epidermis with coarse hyalinized collagen bundles in the mid and lower dermis. There was upper and diffuse dermal elastolysis (patient 1 and patient 2, respectively).¹ Our patient’s lesions were hypopigmented and atrophic in appearance but were slightly scaly and also involved the extremities. Distinct from these patient reports, histopathology from our case demonstrated thin packed collagen bundles and decreased fragmented elastin filaments confined to the upper reticular and papillary dermis.

Plaque morphea is the most common type of localized scleroderma.² The subtype APP demonstrates round to ovoid, gray-brown depressions with cliff-drop borders. They may appear flesh colored or hypopigmented.^3,4 These sclerodermoid lesions lack the violaceous border classic to morphea. Sclerosis and induration also are typically absent.⁵ Clinically, our patient’s macules resembled this entity. Histopathologically, APP shows normal epithelium with an increased basal layer pigmentation; preserved adnexal structures; and mid to lower dermal collagen edema, clumping, and homogenization.^3,4 Elastic fibers classically are unchanged, with exceptions.^6-11 Changes in the collagen and elastin of our patient were unlike those reported in APP, which occur in the mid to lower dermis.

Guttate morphea demonstrates small, pale, minimally indurated, coin-shaped lesions on the trunk. Histopathology reveals less sclerosis and more edema, resembling LS&A.¹² The earliest descriptions of this entity describe 3 stages: ivory/chalk white, scaly, and atrophic. Follicular plugging (absent in this patient) and fine scale can exist at any stage.^13,14 Flattened rete ridges mark an otherwise preserved epidermis; hyalinized collagen typically is superficial and demonstrates less sclerosis yet increased edema.^12-14 Fewer elastic fibers typically are present compared to normal skin. Changes seen in this entity are more superficial, as with our patient, than classic scleroderma. However, classic edema was not found in our patient’s biopsy specimen.

Superficial morphea, occurring predominantly in females, presents with hyperpigmented or hypopigmented patches having minimal to no induration. The lesions typically are asymptomatic. Histopathologically, collagen deposition and inflammation are confined to the superficial dermis without homogenization associated with LS&A, findings that were consistent with this patient’s biopsy.^15,16 However, similar to other morpheaform variants, elastic fibers are unchanged.¹⁵ Verhoeff-van Gieson stain of the biopsy (Figure 3) showed the decreased and fragmented elastin network in the upper reticular and papillary dermis, making this entity less compatible.

Guttate LS&A may present with interfollicular, bluish white macules or papules coalescing into patches or plaques. Lesions evolve to reveal atrophic thin skin with follicular plugging. Histology demonstrates a thinned epidermis with orthohypokeratosis marked by flattened rete ridges. The dermis reveals short hyalinized collagen fibrils with a loss of elastic fibers in the papillary and upper reticular dermis, giving a homogenized appearance. Early disease is marked by an inflammatory infiltrate.¹⁷ Most of these findings are consistent with our patient’s pathology, which was confined to the upper dermis. Lacking, however, were characteristic findings of LS&A, including upper dermal homogenization, near-total effacement of rete ridges, orthokeratosis, and vacuolar degeneration at the dermoepidermal junction. As such, this entity is less compatible.

Atrophoderma elastolyticum discretum has clinical features of atrophoderma with elastolytic histopathologic findings.¹ Anetoderma presents with outpouchings of atrophic skin with a surrounding ring of normal tissue. Histopathologically, this entity shows normal collagen with elastolysis; there also is a decrease in desmosine, an elastin cross-linker.^1,3 Neither the clinical nor histopathologic findings in this patient matched these 2 entities.

The reported chronologic association of these lesions with an arthropod assault raised suspicion to their association with toxic insult or postinflammatory changes. One study reported mechanical trauma, including insect bites, as a possible inciting factor of morphea.¹¹ These data, gathered from patient surveys, reported trauma associated to lesion development.^1,17 A review of the literature regarding atrophoderma, morphea, and LS&A failed to identify pathogenic changes seen in this patient following initial trauma. Moreover, although it is difficult to prove causality in the formation of the original hypopigmented spots, the development of identical spots in a similar distribution without further trauma suggests against these etiologies to fully explain her lesions. Nonetheless, circumstance makes it difficult to prove whether the original arthropod insult spurred a smoldering reactive process that caused the newer lesions.

Hereditary connective-tissue disorders also were considered in the differential diagnosis. Because of the patient’s history of an unprovoked complete rotator cuff tear, Ehlers-Danlos syndrome was considered; however, the remainder of her examination was normal, making a syndromic systemic disorder a less likely etiology.Because of the distinct clinical and histopathologic findings, this case may represent a rare and previously unreported variant of morphea. Clinically, these hypopigmented macules and patches exist somewhere along the morphea-atrophoderma spectrum. Histopathologic findings do not conform to prior reports. The name atrophodermalike guttate morphea may be an appropriate appellation. It is possible this presentation represents a variant of what dermatologists have referred to as white spot disease.¹⁸ We hope that this case may bring others to discussion, allowing for the identification of a more precise entity and etiology so that patients may receive more directed therapy.

To the Editor:

I read with interest the informative article by Sheu¹ published online in Cutis in February 2018, which succinctly described the pharmacologic characteristics of tranexamic acid, a synthetic lysine derivative, and its mechanism of action in the management of melasma by mitigating UV radiation-induced melanogenesis and neovascularization by inhibiting plasminogen activation. Additionally, the author summarized a study in which oral tranexamic acid was used to successfully treat melasma patients. After 4 months of treatment, 90% of 561 patients treated at a single center in Singapore demonstrated improvement in melasma severity.² Sheu¹ also discussed daily oral doses of tranexamic acid (500-1500 mg) that demonstrated improvement in melasma patients and reviewed potential adverse events (eg, abdominal pain and bloating, deep venous thrombosis, pulmonary embolism) for which patients should be screened and counseled prior to initiating treatment.

Recently, another study showed oral tranexamic acid to be an effective treatment in women with moderate to severe melasma. An important observation by the investigators was that once the initial phase of their study--250 mg of oral tranexamic acid twice daily and sunscreen applied to the face each morning and every 2 hours during daylight hours for 3 months--concluded and a second phase during which all participants only applied sunscreen for an additional 3 months, those with severe melasma lost most of their improvement.³ An adjuvant topical treatment, such as tranexamic acid or an inhibitor of tyrosinase (hydroquinone), might improve the results; however, initiating therapy with a topical agent whose mode of action is directed toward other melasma etiologic factors, such as the increased expression of estrogen receptors and vascular endothelial growth factor in affected skin, might be more beneficial.^4,5

I recently proposed a novel approach for melasma management that would be appropriate as an adjuvant topical therapy for patients concurrently being treated with oral tranexamic acid.⁶ The therapeutic intervention utilizes active agents that specifically affect etiologic factors in the pathogenesis of melasma--estrogen and angiogenesis--that previously have not been targeted topically. Indeed, the topical agent contains an antiestrogen--either a selective estrogen receptor modulator (eg, tamoxifen, raloxifene), aromatase inhibitor (eg, anastrozole, letrozole, exemestane), or a selective estrogen receptor degrader (eg, fulvestrant)--and a vascular endothelial growth factor inhibitor (eg, bevacizumab).⁶

In conclusion, the therapeutic armamentarium for managing patients with melasma includes topical agents, oral therapies, and physical modalities. Optimizing the approach to treating melasma patients should incorporate therapies that are specifically directed toward various etiologic factors of the condition. The concurrent use of a topical agent that contains an antiestrogen and an inhibitor of vascular endothelial growth factor in women with melasma who are being treated with oral tranexamic acid warrants further investigation to assess not only for enhanced but also sustained reduction in facial skin pigmentation.

To the Editor:

I read with interest the informative article by Sheu¹ published online in Cutis in February 2018, which succinctly described the pharmacologic characteristics of tranexamic acid, a synthetic lysine derivative, and its mechanism of action in the management of melasma by mitigating UV radiation-induced melanogenesis and neovascularization by inhibiting plasminogen activation. Additionally, the author summarized a study in which oral tranexamic acid was used to successfully treat melasma patients. After 4 months of treatment, 90% of 561 patients treated at a single center in Singapore demonstrated improvement in melasma severity.² Sheu¹ also discussed daily oral doses of tranexamic acid (500-1500 mg) that demonstrated improvement in melasma patients and reviewed potential adverse events (eg, abdominal pain and bloating, deep venous thrombosis, pulmonary embolism) for which patients should be screened and counseled prior to initiating treatment.

Recently, another study showed oral tranexamic acid to be an effective treatment in women with moderate to severe melasma. An important observation by the investigators was that once the initial phase of their study--250 mg of oral tranexamic acid twice daily and sunscreen applied to the face each morning and every 2 hours during daylight hours for 3 months--concluded and a second phase during which all participants only applied sunscreen for an additional 3 months, those with severe melasma lost most of their improvement.³ An adjuvant topical treatment, such as tranexamic acid or an inhibitor of tyrosinase (hydroquinone), might improve the results; however, initiating therapy with a topical agent whose mode of action is directed toward other melasma etiologic factors, such as the increased expression of estrogen receptors and vascular endothelial growth factor in affected skin, might be more beneficial.^4,5

I recently proposed a novel approach for melasma management that would be appropriate as an adjuvant topical therapy for patients concurrently being treated with oral tranexamic acid.⁶ The therapeutic intervention utilizes active agents that specifically affect etiologic factors in the pathogenesis of melasma--estrogen and angiogenesis--that previously have not been targeted topically. Indeed, the topical agent contains an antiestrogen--either a selective estrogen receptor modulator (eg, tamoxifen, raloxifene), aromatase inhibitor (eg, anastrozole, letrozole, exemestane), or a selective estrogen receptor degrader (eg, fulvestrant)--and a vascular endothelial growth factor inhibitor (eg, bevacizumab).⁶

In conclusion, the therapeutic armamentarium for managing patients with melasma includes topical agents, oral therapies, and physical modalities. Optimizing the approach to treating melasma patients should incorporate therapies that are specifically directed toward various etiologic factors of the condition. The concurrent use of a topical agent that contains an antiestrogen and an inhibitor of vascular endothelial growth factor in women with melasma who are being treated with oral tranexamic acid warrants further investigation to assess not only for enhanced but also sustained reduction in facial skin pigmentation.

To the Editor:

I read with interest the informative article by Sheu¹ published online in Cutis in February 2018, which succinctly described the pharmacologic characteristics of tranexamic acid, a synthetic lysine derivative, and its mechanism of action in the management of melasma by mitigating UV radiation-induced melanogenesis and neovascularization by inhibiting plasminogen activation. Additionally, the author summarized a study in which oral tranexamic acid was used to successfully treat melasma patients. After 4 months of treatment, 90% of 561 patients treated at a single center in Singapore demonstrated improvement in melasma severity.² Sheu¹ also discussed daily oral doses of tranexamic acid (500-1500 mg) that demonstrated improvement in melasma patients and reviewed potential adverse events (eg, abdominal pain and bloating, deep venous thrombosis, pulmonary embolism) for which patients should be screened and counseled prior to initiating treatment.

Recently, another study showed oral tranexamic acid to be an effective treatment in women with moderate to severe melasma. An important observation by the investigators was that once the initial phase of their study--250 mg of oral tranexamic acid twice daily and sunscreen applied to the face each morning and every 2 hours during daylight hours for 3 months--concluded and a second phase during which all participants only applied sunscreen for an additional 3 months, those with severe melasma lost most of their improvement.³ An adjuvant topical treatment, such as tranexamic acid or an inhibitor of tyrosinase (hydroquinone), might improve the results; however, initiating therapy with a topical agent whose mode of action is directed toward other melasma etiologic factors, such as the increased expression of estrogen receptors and vascular endothelial growth factor in affected skin, might be more beneficial.^4,5

I recently proposed a novel approach for melasma management that would be appropriate as an adjuvant topical therapy for patients concurrently being treated with oral tranexamic acid.⁶ The therapeutic intervention utilizes active agents that specifically affect etiologic factors in the pathogenesis of melasma--estrogen and angiogenesis--that previously have not been targeted topically. Indeed, the topical agent contains an antiestrogen--either a selective estrogen receptor modulator (eg, tamoxifen, raloxifene), aromatase inhibitor (eg, anastrozole, letrozole, exemestane), or a selective estrogen receptor degrader (eg, fulvestrant)--and a vascular endothelial growth factor inhibitor (eg, bevacizumab).⁶

In conclusion, the therapeutic armamentarium for managing patients with melasma includes topical agents, oral therapies, and physical modalities. Optimizing the approach to treating melasma patients should incorporate therapies that are specifically directed toward various etiologic factors of the condition. The concurrent use of a topical agent that contains an antiestrogen and an inhibitor of vascular endothelial growth factor in women with melasma who are being treated with oral tranexamic acid warrants further investigation to assess not only for enhanced but also sustained reduction in facial skin pigmentation.

LAKE TAHOE, CALIF. – Among children and adolescents, vitiligo appears to predominately affect nonwhite boys and girls between the ages of 10 and 17 years, results from a large cross-sectional analysis demonstrated.

Doug Brunk/MDedge News

During an interview at the annual meeting of the Society for Pediatric Dermatology, lead study author Jessica Haber, MD, said that, while it’s known vitiligo can have its onset in childhood, there have been no population-based analyses in the United States specific to children and adolescents with the condition.

“We wanted to examine disease burden in the U.S. specifically, because we have such a diverse population,” said Dr. Haber, a second-year resident in the department of dermatology at Northwell Health, New York.

For the study, she and her associates used IBM’s Explorys research analytics platform to conduct a cross-sectional analysis of more than 55 million unique patients across all census regions of the United States. There were 1,630 vitiligo cases identified from a total of 4,242,400 pediatric patients, for an overall standard prevalence of 0.04%, or 40.1 per 100,000 children and adolescents. The proportion of female and male patients with vitiligo was similar (49.1% and 50.9%, respectively), and nearly three-fourths (72.3%) were 10 years of age or older.

The researchers observed no significant difference in the prevalence of vitiligo between males and females (40.2 per 100,000 vs. 40 per 100,000, respectively). The standardized prevalence of vitiligo was greatest in pediatric patients who were of “other” races and ethnicities (including Asian, Hispanic, multiracial, and other; 69.1 per 100,000), followed by African Americans (51.5 per 100,000) and whites (37.9 per 100,000). There were too few vitiligo cases among biracial patients to determine standardized estimates, but the crude prevalence was greatest in this group (68.7 per 100,000).

Two factors could contribute to the increased prevalence of vitiligo observed in nonwhite children and adolescents, Dr. Haber said. One is selection bias.

“It has been reported that both children and adults with higher Fitzpatrick skin types tend to have increased morbidity of their vitiligo, so it may be a selection bias that these patients are seeking out treatment for their disease,” she said. “Also, according to recent research in the medical literature, increased melanin production may be a risk factor for the development of vitiligo (J Am Acad Dermatol. 2017;77[1]:1-13). That might explain some of our findings, as well.”

While the study findings “don’t necessarily change clinical practice, it is good for us to have a sense of the burden of disease in the pediatric patient population of vitiligo, and to be aware that this is a disease that predominately affects non-Caucasian children and adolescents,” Dr. Haber concluded.

She reported having no financial disclosures.

[email protected]

LAKE TAHOE, CALIF. – Among children and adolescents, vitiligo appears to predominately affect nonwhite boys and girls between the ages of 10 and 17 years, results from a large cross-sectional analysis demonstrated.

Doug Brunk/MDedge News

During an interview at the annual meeting of the Society for Pediatric Dermatology, lead study author Jessica Haber, MD, said that, while it’s known vitiligo can have its onset in childhood, there have been no population-based analyses in the United States specific to children and adolescents with the condition.

“We wanted to examine disease burden in the U.S. specifically, because we have such a diverse population,” said Dr. Haber, a second-year resident in the department of dermatology at Northwell Health, New York.

For the study, she and her associates used IBM’s Explorys research analytics platform to conduct a cross-sectional analysis of more than 55 million unique patients across all census regions of the United States. There were 1,630 vitiligo cases identified from a total of 4,242,400 pediatric patients, for an overall standard prevalence of 0.04%, or 40.1 per 100,000 children and adolescents. The proportion of female and male patients with vitiligo was similar (49.1% and 50.9%, respectively), and nearly three-fourths (72.3%) were 10 years of age or older.

The researchers observed no significant difference in the prevalence of vitiligo between males and females (40.2 per 100,000 vs. 40 per 100,000, respectively). The standardized prevalence of vitiligo was greatest in pediatric patients who were of “other” races and ethnicities (including Asian, Hispanic, multiracial, and other; 69.1 per 100,000), followed by African Americans (51.5 per 100,000) and whites (37.9 per 100,000). There were too few vitiligo cases among biracial patients to determine standardized estimates, but the crude prevalence was greatest in this group (68.7 per 100,000).

Two factors could contribute to the increased prevalence of vitiligo observed in nonwhite children and adolescents, Dr. Haber said. One is selection bias.

“It has been reported that both children and adults with higher Fitzpatrick skin types tend to have increased morbidity of their vitiligo, so it may be a selection bias that these patients are seeking out treatment for their disease,” she said. “Also, according to recent research in the medical literature, increased melanin production may be a risk factor for the development of vitiligo (J Am Acad Dermatol. 2017;77[1]:1-13). That might explain some of our findings, as well.”

While the study findings “don’t necessarily change clinical practice, it is good for us to have a sense of the burden of disease in the pediatric patient population of vitiligo, and to be aware that this is a disease that predominately affects non-Caucasian children and adolescents,” Dr. Haber concluded.

She reported having no financial disclosures.

[email protected]

LAKE TAHOE, CALIF. – Among children and adolescents, vitiligo appears to predominately affect nonwhite boys and girls between the ages of 10 and 17 years, results from a large cross-sectional analysis demonstrated.

Doug Brunk/MDedge News

During an interview at the annual meeting of the Society for Pediatric Dermatology, lead study author Jessica Haber, MD, said that, while it’s known vitiligo can have its onset in childhood, there have been no population-based analyses in the United States specific to children and adolescents with the condition.

“We wanted to examine disease burden in the U.S. specifically, because we have such a diverse population,” said Dr. Haber, a second-year resident in the department of dermatology at Northwell Health, New York.

For the study, she and her associates used IBM’s Explorys research analytics platform to conduct a cross-sectional analysis of more than 55 million unique patients across all census regions of the United States. There were 1,630 vitiligo cases identified from a total of 4,242,400 pediatric patients, for an overall standard prevalence of 0.04%, or 40.1 per 100,000 children and adolescents. The proportion of female and male patients with vitiligo was similar (49.1% and 50.9%, respectively), and nearly three-fourths (72.3%) were 10 years of age or older.

The researchers observed no significant difference in the prevalence of vitiligo between males and females (40.2 per 100,000 vs. 40 per 100,000, respectively). The standardized prevalence of vitiligo was greatest in pediatric patients who were of “other” races and ethnicities (including Asian, Hispanic, multiracial, and other; 69.1 per 100,000), followed by African Americans (51.5 per 100,000) and whites (37.9 per 100,000). There were too few vitiligo cases among biracial patients to determine standardized estimates, but the crude prevalence was greatest in this group (68.7 per 100,000).

Two factors could contribute to the increased prevalence of vitiligo observed in nonwhite children and adolescents, Dr. Haber said. One is selection bias.

“It has been reported that both children and adults with higher Fitzpatrick skin types tend to have increased morbidity of their vitiligo, so it may be a selection bias that these patients are seeking out treatment for their disease,” she said. “Also, according to recent research in the medical literature, increased melanin production may be a risk factor for the development of vitiligo (J Am Acad Dermatol. 2017;77[1]:1-13). That might explain some of our findings, as well.”

While the study findings “don’t necessarily change clinical practice, it is good for us to have a sense of the burden of disease in the pediatric patient population of vitiligo, and to be aware that this is a disease that predominately affects non-Caucasian children and adolescents,” Dr. Haber concluded.

She reported having no financial disclosures.

[email protected]

The Diagnosis: Corticosteroid-Induced Hypopigmentation

This patient received several intralesional injections of triamcinolone acetonide once monthly for treatment of the keloid scar on the left ear at an outside institution. There was improvement in the size of the keloid over time. On physical examination during the most recent visit there was a prominent streak of hypopigmentation and atrophy near the corticosteroid injection site with extension to the postauricular region. There also was telangiectasia noted within the area of hypopigmentation. Intralesional triamcinolone injections were discontinued and the patient was advised to return for monitoring.

Intra-articular and intralesional corticosteroid injections frequently are used by clinicians. Cutaneous complications associated with these injections include atrophy, pigmentary changes, hypersensitivity reactions, flushing, cellulitis, and necrotizing fasciitis. Tendon rupture also has been reported.¹

There are several case reports in the literature describing hypopigmentation and/or subcutaneous atrophy after intralesional or intra-articular corticosteroid injections. A variety of underlying conditions were treated including alopecia areata, keloids, rheumatoid arthritis, de Quervain tendonitis, and psoriasis.^2-6 The lesions typically are described as linear rays of atrophy and hypopigmentation at or near the injection site, with some cases noting extension along lymph channels and proximal veins.^4,6 There usually is no associated pruritus or pain.³ This phenomenon can be seen after single or multiple injections.^4,6

Extension of hypopigmentation from the site of injection has been postulated to be due to venous or lymphatic uptake.^2,4-6 The mechanism of hypopigmentation is not known. Biopsy of a previously described case showed intact melanocytes along the dermoepidermal junction.² Biopsy from another case revealed a decrease in melanin staining, which suggests a decrease in number or activity of melanocytes.⁴ It was proposed that hypopigmentation was secondary to loss of melanocyte function instead of loss of melanocytes.² Spontaneous improvement or resolution of the hypopigmentation were noted in some cases ranging from 1 month to 1 year after initial presentation, but the hypopigmentation also can be persistent.^3-6

Hypopigmented sarcoidosis and hypopigmented mycosis fungoides, both often present on dark-skinned individuals, are included in the differential diagnosis. Hypopigmented sarcoidosis presents with hypopigmented macules or patches, some with central papules, and hypopigmented mycosis fungoides presents with hypopigmented patches or plaques with fine scale and onset often in childhood or adolescence.^7,8 Morphea can present with an initial inflammatory stage that develops into a sclerotic firm plaque or nodule with hyperpigmentation or hypopigmentation.⁹ Vitiligo usually presents with depigmented macules or patches and depigmented hair within the lesion.¹⁰

The Diagnosis: Corticosteroid-Induced Hypopigmentation

This patient received several intralesional injections of triamcinolone acetonide once monthly for treatment of the keloid scar on the left ear at an outside institution. There was improvement in the size of the keloid over time. On physical examination during the most recent visit there was a prominent streak of hypopigmentation and atrophy near the corticosteroid injection site with extension to the postauricular region. There also was telangiectasia noted within the area of hypopigmentation. Intralesional triamcinolone injections were discontinued and the patient was advised to return for monitoring.

Intra-articular and intralesional corticosteroid injections frequently are used by clinicians. Cutaneous complications associated with these injections include atrophy, pigmentary changes, hypersensitivity reactions, flushing, cellulitis, and necrotizing fasciitis. Tendon rupture also has been reported.¹

There are several case reports in the literature describing hypopigmentation and/or subcutaneous atrophy after intralesional or intra-articular corticosteroid injections. A variety of underlying conditions were treated including alopecia areata, keloids, rheumatoid arthritis, de Quervain tendonitis, and psoriasis.^2-6 The lesions typically are described as linear rays of atrophy and hypopigmentation at or near the injection site, with some cases noting extension along lymph channels and proximal veins.^4,6 There usually is no associated pruritus or pain.³ This phenomenon can be seen after single or multiple injections.^4,6

Extension of hypopigmentation from the site of injection has been postulated to be due to venous or lymphatic uptake.^2,4-6 The mechanism of hypopigmentation is not known. Biopsy of a previously described case showed intact melanocytes along the dermoepidermal junction.² Biopsy from another case revealed a decrease in melanin staining, which suggests a decrease in number or activity of melanocytes.⁴ It was proposed that hypopigmentation was secondary to loss of melanocyte function instead of loss of melanocytes.² Spontaneous improvement or resolution of the hypopigmentation were noted in some cases ranging from 1 month to 1 year after initial presentation, but the hypopigmentation also can be persistent.^3-6

Hypopigmented sarcoidosis and hypopigmented mycosis fungoides, both often present on dark-skinned individuals, are included in the differential diagnosis. Hypopigmented sarcoidosis presents with hypopigmented macules or patches, some with central papules, and hypopigmented mycosis fungoides presents with hypopigmented patches or plaques with fine scale and onset often in childhood or adolescence.^7,8 Morphea can present with an initial inflammatory stage that develops into a sclerotic firm plaque or nodule with hyperpigmentation or hypopigmentation.⁹ Vitiligo usually presents with depigmented macules or patches and depigmented hair within the lesion.¹⁰

The Diagnosis: Corticosteroid-Induced Hypopigmentation

This patient received several intralesional injections of triamcinolone acetonide once monthly for treatment of the keloid scar on the left ear at an outside institution. There was improvement in the size of the keloid over time. On physical examination during the most recent visit there was a prominent streak of hypopigmentation and atrophy near the corticosteroid injection site with extension to the postauricular region. There also was telangiectasia noted within the area of hypopigmentation. Intralesional triamcinolone injections were discontinued and the patient was advised to return for monitoring.

Intra-articular and intralesional corticosteroid injections frequently are used by clinicians. Cutaneous complications associated with these injections include atrophy, pigmentary changes, hypersensitivity reactions, flushing, cellulitis, and necrotizing fasciitis. Tendon rupture also has been reported.¹

There are several case reports in the literature describing hypopigmentation and/or subcutaneous atrophy after intralesional or intra-articular corticosteroid injections. A variety of underlying conditions were treated including alopecia areata, keloids, rheumatoid arthritis, de Quervain tendonitis, and psoriasis.^2-6 The lesions typically are described as linear rays of atrophy and hypopigmentation at or near the injection site, with some cases noting extension along lymph channels and proximal veins.^4,6 There usually is no associated pruritus or pain.³ This phenomenon can be seen after single or multiple injections.^4,6

Extension of hypopigmentation from the site of injection has been postulated to be due to venous or lymphatic uptake.^2,4-6 The mechanism of hypopigmentation is not known. Biopsy of a previously described case showed intact melanocytes along the dermoepidermal junction.² Biopsy from another case revealed a decrease in melanin staining, which suggests a decrease in number or activity of melanocytes.⁴ It was proposed that hypopigmentation was secondary to loss of melanocyte function instead of loss of melanocytes.² Spontaneous improvement or resolution of the hypopigmentation were noted in some cases ranging from 1 month to 1 year after initial presentation, but the hypopigmentation also can be persistent.^3-6

Hypopigmented sarcoidosis and hypopigmented mycosis fungoides, both often present on dark-skinned individuals, are included in the differential diagnosis. Hypopigmented sarcoidosis presents with hypopigmented macules or patches, some with central papules, and hypopigmented mycosis fungoides presents with hypopigmented patches or plaques with fine scale and onset often in childhood or adolescence.^7,8 Morphea can present with an initial inflammatory stage that develops into a sclerotic firm plaque or nodule with hyperpigmentation or hypopigmentation.⁹ Vitiligo usually presents with depigmented macules or patches and depigmented hair within the lesion.¹⁰

LAKE TAHOE, CALIF. – Children with large facial hemangiomas who have S1 involvement, a lesion area greater than 25 cm², or bilateral location should be prioritized for PHACE syndrome work-up.

In addition, children with isolated S2 or parotid hemangiomas should be recognized as having lower risk for PHACE, and specifics of evaluation should be discussed with parents on a case-by-case basis.

Those are key findings from a retrospective cohort study presented by Colleen Cotton, MD, at the annual meeting of the Society for Pediatric Dermatology.

An association between large facial hemangiomas and multiple abnormalities was described as early as 1978, but it wasn’t until 1996 that researchers first proposed the term PHACE to describe the association (Arch Dermatol. 1996;132[3]:307-11). As the National Institutes of Health explain, “PHACE is an acronym for a neurocutaneous syndrome encompassing the following features: posterior fossa brain malformations, hemangiomas of the face, arterial anomalies, cardiac anomalies, and eye abnormalities.” Official diagnostic criteria for PHACE were not established until 2009 (Pediatrics. 2009;124[5]:1447-56) and were updated in 2016 (J Pediatr. 2016;178:24-33.e2).

“A multicenter, prospective, cohort study published in 2010 estimated the incidence of PHACE to be 31% in patients with large facial hemangiomas, while a retrospective study published in 2017 estimated the incidence to be as high as 58%,” Dr. Cotton, chief dermatology resident at the University of Arizona, Tucson, said in an interview in advance of the meeting. “With the current understanding of risk for PHACE, any child with a facial hemangioma of greater than or equal to 5 cm in diameter receives a full work-up for the syndrome. However, there has been anecdotal evidence that patients with certain subtypes of hemangiomas (such as parotid hemangiomas) may not carry this same risk.”

In what is believed to be the largest study of its kind, Dr. Cotton and her associates retrospectively analyzed data from 244 patients from 13 pediatric dermatology centers who were fully evaluated for PHACE between August 2009 and December 2014. The investigators also performed subgroup analyses on different hemangioma characteristics, including parotid hemangiomas and specific facial segments of involvement. All patients underwent magnetic resonance imaging/magnetic resonance angiography of the head and neck, and the researchers collected data on age at diagnosis; gender; patterns of hemangioma presentation, including location, size, and depth; diagnostic procedures and results; and type and number of associated anomalies. An expert reviewed photographs or diagrams to confirm facial segment locations.

Of the 244 patients, 34.7% met criteria for PHACE syndrome. On multivariate analysis, the following factors were found to be independently and significantly associated with a risk for PHACE: bilateral location (positive predictive value, 54.9%), S1 involvement (PPV, 49.5%), S3 involvement (PPV, 39.5%), and area greater than 25cm² (PPV, 44.8%), with a P value less than .05 for all associations.

Risk of PHACE also increased with the number of locations involved, with a sharp increase observed at three or more locations (PPV, 65.5%; P less than .001). In patients with one unilateral segment involved, S2 and S3 carried a significantly lower risk (P less than .03). Parotid hemangiomas had a negative predictive value of 80.4% (P = .035).

“While we found that patients with parotid hemangiomas had a lower risk of PHACE, 10 patients with parotid hemangiomas did have PHACE, and 90% of those patients had cerebral arterial anomalies,” Dr. Cotton said. “However, only one of these patients had an isolated unilateral parotid hemangioma without other facial segment involvement. Additionally, two patients with isolated involvement of the midcheek below the eye [the S2 location, which was another low risk segment] also had PHACE, both of whom would have been missed without MRI/MRA [magnetic resonance angiography].”

She acknowledged certain limitations of the study, including its retrospective design. “Additionally, many of the very large hemangiomas were not measured in size, and so, estimated sizes needed to be used in calculating relationship of hemangioma size with risk of PHACE,” she said.

The study was funded in part by a grant from the Pediatric Dermatology Research Alliance.* Dr. Cotton reported having no relevant financial disclosures.

[email protected]

Correction, 7/20/18: An earlier version of this article misstated the name of the Pediatric Dermatology Research Alliance.

LAKE TAHOE, CALIF. – Children with large facial hemangiomas who have S1 involvement, a lesion area greater than 25 cm², or bilateral location should be prioritized for PHACE syndrome work-up.

In addition, children with isolated S2 or parotid hemangiomas should be recognized as having lower risk for PHACE, and specifics of evaluation should be discussed with parents on a case-by-case basis.

Those are key findings from a retrospective cohort study presented by Colleen Cotton, MD, at the annual meeting of the Society for Pediatric Dermatology.

An association between large facial hemangiomas and multiple abnormalities was described as early as 1978, but it wasn’t until 1996 that researchers first proposed the term PHACE to describe the association (Arch Dermatol. 1996;132[3]:307-11). As the National Institutes of Health explain, “PHACE is an acronym for a neurocutaneous syndrome encompassing the following features: posterior fossa brain malformations, hemangiomas of the face, arterial anomalies, cardiac anomalies, and eye abnormalities.” Official diagnostic criteria for PHACE were not established until 2009 (Pediatrics. 2009;124[5]:1447-56) and were updated in 2016 (J Pediatr. 2016;178:24-33.e2).

“A multicenter, prospective, cohort study published in 2010 estimated the incidence of PHACE to be 31% in patients with large facial hemangiomas, while a retrospective study published in 2017 estimated the incidence to be as high as 58%,” Dr. Cotton, chief dermatology resident at the University of Arizona, Tucson, said in an interview in advance of the meeting. “With the current understanding of risk for PHACE, any child with a facial hemangioma of greater than or equal to 5 cm in diameter receives a full work-up for the syndrome. However, there has been anecdotal evidence that patients with certain subtypes of hemangiomas (such as parotid hemangiomas) may not carry this same risk.”

In what is believed to be the largest study of its kind, Dr. Cotton and her associates retrospectively analyzed data from 244 patients from 13 pediatric dermatology centers who were fully evaluated for PHACE between August 2009 and December 2014. The investigators also performed subgroup analyses on different hemangioma characteristics, including parotid hemangiomas and specific facial segments of involvement. All patients underwent magnetic resonance imaging/magnetic resonance angiography of the head and neck, and the researchers collected data on age at diagnosis; gender; patterns of hemangioma presentation, including location, size, and depth; diagnostic procedures and results; and type and number of associated anomalies. An expert reviewed photographs or diagrams to confirm facial segment locations.

Of the 244 patients, 34.7% met criteria for PHACE syndrome. On multivariate analysis, the following factors were found to be independently and significantly associated with a risk for PHACE: bilateral location (positive predictive value, 54.9%), S1 involvement (PPV, 49.5%), S3 involvement (PPV, 39.5%), and area greater than 25cm² (PPV, 44.8%), with a P value less than .05 for all associations.

Risk of PHACE also increased with the number of locations involved, with a sharp increase observed at three or more locations (PPV, 65.5%; P less than .001). In patients with one unilateral segment involved, S2 and S3 carried a significantly lower risk (P less than .03). Parotid hemangiomas had a negative predictive value of 80.4% (P = .035).

“While we found that patients with parotid hemangiomas had a lower risk of PHACE, 10 patients with parotid hemangiomas did have PHACE, and 90% of those patients had cerebral arterial anomalies,” Dr. Cotton said. “However, only one of these patients had an isolated unilateral parotid hemangioma without other facial segment involvement. Additionally, two patients with isolated involvement of the midcheek below the eye [the S2 location, which was another low risk segment] also had PHACE, both of whom would have been missed without MRI/MRA [magnetic resonance angiography].”

She acknowledged certain limitations of the study, including its retrospective design. “Additionally, many of the very large hemangiomas were not measured in size, and so, estimated sizes needed to be used in calculating relationship of hemangioma size with risk of PHACE,” she said.

The study was funded in part by a grant from the Pediatric Dermatology Research Alliance.* Dr. Cotton reported having no relevant financial disclosures.

[email protected]

Correction, 7/20/18: An earlier version of this article misstated the name of the Pediatric Dermatology Research Alliance.

LAKE TAHOE, CALIF. – Children with large facial hemangiomas who have S1 involvement, a lesion area greater than 25 cm², or bilateral location should be prioritized for PHACE syndrome work-up.

In addition, children with isolated S2 or parotid hemangiomas should be recognized as having lower risk for PHACE, and specifics of evaluation should be discussed with parents on a case-by-case basis.

Those are key findings from a retrospective cohort study presented by Colleen Cotton, MD, at the annual meeting of the Society for Pediatric Dermatology.

An association between large facial hemangiomas and multiple abnormalities was described as early as 1978, but it wasn’t until 1996 that researchers first proposed the term PHACE to describe the association (Arch Dermatol. 1996;132[3]:307-11). As the National Institutes of Health explain, “PHACE is an acronym for a neurocutaneous syndrome encompassing the following features: posterior fossa brain malformations, hemangiomas of the face, arterial anomalies, cardiac anomalies, and eye abnormalities.” Official diagnostic criteria for PHACE were not established until 2009 (Pediatrics. 2009;124[5]:1447-56) and were updated in 2016 (J Pediatr. 2016;178:24-33.e2).

“A multicenter, prospective, cohort study published in 2010 estimated the incidence of PHACE to be 31% in patients with large facial hemangiomas, while a retrospective study published in 2017 estimated the incidence to be as high as 58%,” Dr. Cotton, chief dermatology resident at the University of Arizona, Tucson, said in an interview in advance of the meeting. “With the current understanding of risk for PHACE, any child with a facial hemangioma of greater than or equal to 5 cm in diameter receives a full work-up for the syndrome. However, there has been anecdotal evidence that patients with certain subtypes of hemangiomas (such as parotid hemangiomas) may not carry this same risk.”

In what is believed to be the largest study of its kind, Dr. Cotton and her associates retrospectively analyzed data from 244 patients from 13 pediatric dermatology centers who were fully evaluated for PHACE between August 2009 and December 2014. The investigators also performed subgroup analyses on different hemangioma characteristics, including parotid hemangiomas and specific facial segments of involvement. All patients underwent magnetic resonance imaging/magnetic resonance angiography of the head and neck, and the researchers collected data on age at diagnosis; gender; patterns of hemangioma presentation, including location, size, and depth; diagnostic procedures and results; and type and number of associated anomalies. An expert reviewed photographs or diagrams to confirm facial segment locations.

Of the 244 patients, 34.7% met criteria for PHACE syndrome. On multivariate analysis, the following factors were found to be independently and significantly associated with a risk for PHACE: bilateral location (positive predictive value, 54.9%), S1 involvement (PPV, 49.5%), S3 involvement (PPV, 39.5%), and area greater than 25cm² (PPV, 44.8%), with a P value less than .05 for all associations.

Risk of PHACE also increased with the number of locations involved, with a sharp increase observed at three or more locations (PPV, 65.5%; P less than .001). In patients with one unilateral segment involved, S2 and S3 carried a significantly lower risk (P less than .03). Parotid hemangiomas had a negative predictive value of 80.4% (P = .035).

“While we found that patients with parotid hemangiomas had a lower risk of PHACE, 10 patients with parotid hemangiomas did have PHACE, and 90% of those patients had cerebral arterial anomalies,” Dr. Cotton said. “However, only one of these patients had an isolated unilateral parotid hemangioma without other facial segment involvement. Additionally, two patients with isolated involvement of the midcheek below the eye [the S2 location, which was another low risk segment] also had PHACE, both of whom would have been missed without MRI/MRA [magnetic resonance angiography].”

She acknowledged certain limitations of the study, including its retrospective design. “Additionally, many of the very large hemangiomas were not measured in size, and so, estimated sizes needed to be used in calculating relationship of hemangioma size with risk of PHACE,” she said.

The study was funded in part by a grant from the Pediatric Dermatology Research Alliance.* Dr. Cotton reported having no relevant financial disclosures.

[email protected]

Correction, 7/20/18: An earlier version of this article misstated the name of the Pediatric Dermatology Research Alliance.

To the Editor:

A 13-year-old white adolescent girl presented with asymptomatic discrete hyperpigmented papules on the chest, back, arms, and upper legs of 7 months’ duration. The patient otherwise was in good health; her weight and height were on the 40th percentile on growth curves and she had no history of any medications. Treatments for the skin condition prescribed by outside dermatologists included minocycline 75 mg twice daily for 2 months, lactic acid lotion 12% daily, and ketoconazole 400 mg administered twice 1 week apart.

Physical examination revealed more than 50 scattered hyperpigmented papules on the chest, back, arms, and upper legs ranging in size from 2 to 3.5 cm (Figure 1). Stroking of lesions failed to elicit Darier sign. A potassium hydroxide preparation and fungal culture were negative for pathogenic fungal organisms. The plasma insulin level was within reference range. A punch biopsy from the abdomen was obtained and sent for histopathologic examination. Histopathology showed mild hyperkeratosis, subtle papillomatosis, and interanastomosing acanthosis comprising squamoid cells with mild basilar hyperpigmentation (Figure 2). Sparse superficial perivascular lymphocytic infiltrate and increased pigmentation was seen in the basal layer. The dermis showed a few scattered dermal melanophages. A periodic acid–Schiff with diastase stain was negative. Giemsa and Leder stains highlighted a normal number and distribution of mast cells. Based on the histologic findings, the patient was diagnosed with idiopathic eruptive macular pigmentation (IEMP).

Idiopathic eruptive macular pigmentation is a rare condition that was described in 1978 by Degos et al.¹ Sanz de Galdeano et al² established the following diagnostic criteria: (1) eruption of brownish black, nonconfluent, asymptomatic macules involving the trunk, neck, and proximal arms and legs in children or adolescents; (2) absence of preceding inflammatory lesions; (3) no prior drug exposure; (4) basal cell layer hyperpigmentation of the epidermis and prominent dermal melanophages without visible basal layer damage or lichenoid inflammatory infiltrate; and (5) normal mast cell count.

Idiopathic eruptive macular pigmentation with papillomatosis (IEMPwP) is a variant of IEMP.³ It is undecided if IEMP and IEMPwP are variants of the same entity or distinct conditions. Until a clear etiology of these entities is established, we prefer to separate them on purely morphologic grounds. Marcoux et al⁴ labeled IEMPwP as a variant of acanthosis nigricans. Although morphologically the 2 conditions appear similar, our patient’s plasma insulin level essentially ruled out acanthosis nigricans.

Idiopathic eruptive macular pigmentation is a rare condition with the majority of cases reported in the Asian population with some reports in white, Hispanic, and black individuals.⁵ Idiopathic eruptive macular pigmentation with papillomatosis was reported by Joshi³ in 2007 in 9 Indian children with the classic findings of IEMP along with a velvety rash that correlated with papillomatosis. Diagnosis of IEMPwP is important, as the disease generally is self-limited and resolves over the course of a few weeks to a few years.

To the Editor:

A 13-year-old white adolescent girl presented with asymptomatic discrete hyperpigmented papules on the chest, back, arms, and upper legs of 7 months’ duration. The patient otherwise was in good health; her weight and height were on the 40th percentile on growth curves and she had no history of any medications. Treatments for the skin condition prescribed by outside dermatologists included minocycline 75 mg twice daily for 2 months, lactic acid lotion 12% daily, and ketoconazole 400 mg administered twice 1 week apart.

Physical examination revealed more than 50 scattered hyperpigmented papules on the chest, back, arms, and upper legs ranging in size from 2 to 3.5 cm (Figure 1). Stroking of lesions failed to elicit Darier sign. A potassium hydroxide preparation and fungal culture were negative for pathogenic fungal organisms. The plasma insulin level was within reference range. A punch biopsy from the abdomen was obtained and sent for histopathologic examination. Histopathology showed mild hyperkeratosis, subtle papillomatosis, and interanastomosing acanthosis comprising squamoid cells with mild basilar hyperpigmentation (Figure 2). Sparse superficial perivascular lymphocytic infiltrate and increased pigmentation was seen in the basal layer. The dermis showed a few scattered dermal melanophages. A periodic acid–Schiff with diastase stain was negative. Giemsa and Leder stains highlighted a normal number and distribution of mast cells. Based on the histologic findings, the patient was diagnosed with idiopathic eruptive macular pigmentation (IEMP).

Idiopathic eruptive macular pigmentation is a rare condition that was described in 1978 by Degos et al.¹ Sanz de Galdeano et al² established the following diagnostic criteria: (1) eruption of brownish black, nonconfluent, asymptomatic macules involving the trunk, neck, and proximal arms and legs in children or adolescents; (2) absence of preceding inflammatory lesions; (3) no prior drug exposure; (4) basal cell layer hyperpigmentation of the epidermis and prominent dermal melanophages without visible basal layer damage or lichenoid inflammatory infiltrate; and (5) normal mast cell count.

Idiopathic eruptive macular pigmentation with papillomatosis (IEMPwP) is a variant of IEMP.³ It is undecided if IEMP and IEMPwP are variants of the same entity or distinct conditions. Until a clear etiology of these entities is established, we prefer to separate them on purely morphologic grounds. Marcoux et al⁴ labeled IEMPwP as a variant of acanthosis nigricans. Although morphologically the 2 conditions appear similar, our patient’s plasma insulin level essentially ruled out acanthosis nigricans.

Idiopathic eruptive macular pigmentation is a rare condition with the majority of cases reported in the Asian population with some reports in white, Hispanic, and black individuals.⁵ Idiopathic eruptive macular pigmentation with papillomatosis was reported by Joshi³ in 2007 in 9 Indian children with the classic findings of IEMP along with a velvety rash that correlated with papillomatosis. Diagnosis of IEMPwP is important, as the disease generally is self-limited and resolves over the course of a few weeks to a few years.

To the Editor:

A 13-year-old white adolescent girl presented with asymptomatic discrete hyperpigmented papules on the chest, back, arms, and upper legs of 7 months’ duration. The patient otherwise was in good health; her weight and height were on the 40th percentile on growth curves and she had no history of any medications. Treatments for the skin condition prescribed by outside dermatologists included minocycline 75 mg twice daily for 2 months, lactic acid lotion 12% daily, and ketoconazole 400 mg administered twice 1 week apart.

Physical examination revealed more than 50 scattered hyperpigmented papules on the chest, back, arms, and upper legs ranging in size from 2 to 3.5 cm (Figure 1). Stroking of lesions failed to elicit Darier sign. A potassium hydroxide preparation and fungal culture were negative for pathogenic fungal organisms. The plasma insulin level was within reference range. A punch biopsy from the abdomen was obtained and sent for histopathologic examination. Histopathology showed mild hyperkeratosis, subtle papillomatosis, and interanastomosing acanthosis comprising squamoid cells with mild basilar hyperpigmentation (Figure 2). Sparse superficial perivascular lymphocytic infiltrate and increased pigmentation was seen in the basal layer. The dermis showed a few scattered dermal melanophages. A periodic acid–Schiff with diastase stain was negative. Giemsa and Leder stains highlighted a normal number and distribution of mast cells. Based on the histologic findings, the patient was diagnosed with idiopathic eruptive macular pigmentation (IEMP).

Idiopathic eruptive macular pigmentation is a rare condition that was described in 1978 by Degos et al.¹ Sanz de Galdeano et al² established the following diagnostic criteria: (1) eruption of brownish black, nonconfluent, asymptomatic macules involving the trunk, neck, and proximal arms and legs in children or adolescents; (2) absence of preceding inflammatory lesions; (3) no prior drug exposure; (4) basal cell layer hyperpigmentation of the epidermis and prominent dermal melanophages without visible basal layer damage or lichenoid inflammatory infiltrate; and (5) normal mast cell count.

Idiopathic eruptive macular pigmentation with papillomatosis (IEMPwP) is a variant of IEMP.³ It is undecided if IEMP and IEMPwP are variants of the same entity or distinct conditions. Until a clear etiology of these entities is established, we prefer to separate them on purely morphologic grounds. Marcoux et al⁴ labeled IEMPwP as a variant of acanthosis nigricans. Although morphologically the 2 conditions appear similar, our patient’s plasma insulin level essentially ruled out acanthosis nigricans.

Idiopathic eruptive macular pigmentation is a rare condition with the majority of cases reported in the Asian population with some reports in white, Hispanic, and black individuals.⁵ Idiopathic eruptive macular pigmentation with papillomatosis was reported by Joshi³ in 2007 in 9 Indian children with the classic findings of IEMP along with a velvety rash that correlated with papillomatosis. Diagnosis of IEMPwP is important, as the disease generally is self-limited and resolves over the course of a few weeks to a few years.