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Erythrokeratoderma variabilis in a Native American
P3009
P3011
Outpatient visits by children with psoriasis in the United States: 1979 to 2007 Sinae Vogel, UCSF, San Francisco, CA, United States; Alan Fleischer, MD, Wake Forest University, Winston-Salem, NC, United States; Brad Yentzer, MD, Wake Forest University, Winston-Salem, NC, United States; Kelly Cordoro, MD, UCSF, San Francisco, CA, United States; Steven Feldman, MD, PhD, Wake Forest University, Winston-Salem, NC, United States Background: Management of pediatric psoriasis varies by patient age group and medical specialty.
Erythrokeratoderma variabilis in a Native American Erica Walsh, MD, University of New Mexico, Albuquerque, NM, United States; Aimee Smidt, MD, University of New Mexico, Albuquerque, NM, United States; Barrett Zlotoff, MD, University of New Mexico, Albuquerque, NM, United States A 3-year-old Native American male presented with a 2-year history of symmetric, erythematous, ichthyotic, pruritic patches and plaques exacerbated by cold temperature. The plaques reportedly begin in different places on his body and have borders that seem to change and expand over a period of a day or two. He was previously diagnosed with atopic dermatitis that was unresponsive to triamcinolone and pimecrolimus. On examination there were well demarcated, erythematous patches and plaques with geometric borders and fine overlying scale in a symmetrical distribution on the cheeks, anterior neck, presternal chest, upper arms and lower legs, and buttocks. A biopsy specimen obtained from the left buttock revealed slight orthohyperkeratosis with mild papillomatosis and mildly thickened granular layer. He was diagnosed with erythrokeratoderma variabilis and topical tazarotene 0.1% gel was initiated. Erythrokeratoderma variabilis is a rare autosomal dominant genodermatosis with approximately 200 cases reported thus far. A majority of cases affect whites. This is the first case of a Native American with erythrokeratoderma variabilis reported in the literature.
Objective: Characterize patterns of childhood psoriasis health care delivery from 1979-2007. Design: Retrospective, cross-sectional investigation utilizing National Ambulatory Medical Care Survey data (NAMCS). Setting: US ambulatory physician offices during years 1979-81, 1986 and 1989-2007. Patients: Psoriatic children ages 0-18. Main outcome measures: Demographics, provider specialty, and medications listed. Results: More than 3.9 million visits for psoriasis were documented over a 23-year period with a median of 123,420 visits per year. Visits to dermatologists comprised 63%, pediatricians 17%, and internists 14%. Number of visits were equal between genders but ranged by age group with ages 13 to 18 accounting for 47% of visits, ages 8 to 12 with 35%, and ages 0 to 7 with 18%. Ninety-three percent of patients were white; 85% were non-Hispanic. Topical corticosteroids were most commonly prescribed, and children 0 to 9 years received equally potent agents as children 10 to 18 years with an average potency class of 4.0 and 3.5, respectively. Among all patients, the most listed medication was topical betamethasone; among ages 0 to 9, tacrolimus; and among ages 10 to 18, betamethasone. By specialty, the most listed medications were fluocinonide (dermatology), tacrolimus (pediatrics), and betamethasone (internal medicine). There were no observed prescriptions for topical calcineurin inhibitors by dermatologists and systemic antipsoriatic agents were not among the top 20 most listed medications in any age group.
Commercial support: None identified.
Conclusions: Over a 23-year period in the US, office visits for psoriasis were attended mostly by white children older than 8 years with equal gender distribution. Dermatologists and pediatricians are the primary caregivers and treatment differs between these two provider groups and among children of different ages. Commercial support: None identified.
P3012 A case of benign cephalic histiocytosis with clinical and histologic features of juvenile xanthogranuloma on follow-up: Additional support for a unifying concept L. Katie Morrison, MD, University of Texas Houston, Houston, TX, United States
P3010
The etiology of histiocytic and nonhistiocytic disorders is poorly understood. NonLangerhans histiocyte (non-LCH) disorders are a group of conditions characterized by abnormal histiocytic proliferations of the non-Langerhans type. These include benign cephalic histiocytosis (BCH), juvenile xanthogranuloma (JXG), and generalized eruptive histiocytosis (GEH). A black female with a history of BCH diagnosed at 3 months of age on clinical, histologic, and immunohistochemical profiles presented for a follow-up 5 months after diagnosis. New yellowish-brown nodules were now visible, as well as the progression of the previous papular lesions into dome shaped nodules. One of these, a 1-cm nodule, was noted at the left lateral epicanthus. Also evident was the progression of some of the previous papules into multilobular nodular plaques. Her physical examination was otherwise normal. A biopsy specimen from a multilobulated plaque revealed a well-circumscribed, dense histiocytic infiltrate in the superficial dermis. Multinucleated giant cells forming nuclear wreath shapes were present, which were not visible on biopsy at 3 months of age. No foamy histiocytes were seen. Immunohistochemistry was strongly positivity for CD68 and negative for CD1a. These findings are characteristic of JXG. On ophthalmology examination there was no ocular involvement. This case clinically and histologically supports the related spectrum of BCH and JXG, which has been published from the same patient at different time points only one other time. Of note in this case are the clinical changes that prompted the additional biopsy specimen. This may indicate that cases of BCH that will subsequently demonstrate features of JXG will also have clinical changes. A multilobular nodular plaque was chosen for biopsy in this infant because of its easily accessible and cosmetically acceptable location on the posterior torso. Because there were a few distinct appearing cutaneous plaques and nodules present at the second biopsy, it is hypothesized here that a biopsy obtained from one of the larger nodular lesions would have demonstrated a more mature form of JXG with more Touton cells and foamy histiocytes. Further studies are indicated to better understand the etiology of these non-LCH disorders and their relationships. Commercial support: None identified.
FEBRUARY 2011
J AM ACAD DERMATOL
AB131
P3011
Outpatient visits by children with psoriasis in the United States: 1979 to 2007 Sinae Vogel, UCSF, San Francisco, CA, United States; Alan Fleischer, MD, Wake Forest University, Winston-Salem, NC, United States; Brad Yentzer, MD, Wake Forest University, Winston-Salem, NC, United States; Kelly Cordoro, MD, UCSF, San Francisco, CA, United States; Steven Feldman, MD, PhD, Wake Forest University, Winston-Salem, NC, United States Background: Management of pediatric psoriasis varies by patient age group and medical specialty.
Erythrokeratoderma variabilis in a Native American Erica Walsh, MD, University of New Mexico, Albuquerque, NM, United States; Aimee Smidt, MD, University of New Mexico, Albuquerque, NM, United States; Barrett Zlotoff, MD, University of New Mexico, Albuquerque, NM, United States A 3-year-old Native American male presented with a 2-year history of symmetric, erythematous, ichthyotic, pruritic patches and plaques exacerbated by cold temperature. The plaques reportedly begin in different places on his body and have borders that seem to change and expand over a period of a day or two. He was previously diagnosed with atopic dermatitis that was unresponsive to triamcinolone and pimecrolimus. On examination there were well demarcated, erythematous patches and plaques with geometric borders and fine overlying scale in a symmetrical distribution on the cheeks, anterior neck, presternal chest, upper arms and lower legs, and buttocks. A biopsy specimen obtained from the left buttock revealed slight orthohyperkeratosis with mild papillomatosis and mildly thickened granular layer. He was diagnosed with erythrokeratoderma variabilis and topical tazarotene 0.1% gel was initiated. Erythrokeratoderma variabilis is a rare autosomal dominant genodermatosis with approximately 200 cases reported thus far. A majority of cases affect whites. This is the first case of a Native American with erythrokeratoderma variabilis reported in the literature.
Objective: Characterize patterns of childhood psoriasis health care delivery from 1979-2007. Design: Retrospective, cross-sectional investigation utilizing National Ambulatory Medical Care Survey data (NAMCS). Setting: US ambulatory physician offices during years 1979-81, 1986 and 1989-2007. Patients: Psoriatic children ages 0-18. Main outcome measures: Demographics, provider specialty, and medications listed. Results: More than 3.9 million visits for psoriasis were documented over a 23-year period with a median of 123,420 visits per year. Visits to dermatologists comprised 63%, pediatricians 17%, and internists 14%. Number of visits were equal between genders but ranged by age group with ages 13 to 18 accounting for 47% of visits, ages 8 to 12 with 35%, and ages 0 to 7 with 18%. Ninety-three percent of patients were white; 85% were non-Hispanic. Topical corticosteroids were most commonly prescribed, and children 0 to 9 years received equally potent agents as children 10 to 18 years with an average potency class of 4.0 and 3.5, respectively. Among all patients, the most listed medication was topical betamethasone; among ages 0 to 9, tacrolimus; and among ages 10 to 18, betamethasone. By specialty, the most listed medications were fluocinonide (dermatology), tacrolimus (pediatrics), and betamethasone (internal medicine). There were no observed prescriptions for topical calcineurin inhibitors by dermatologists and systemic antipsoriatic agents were not among the top 20 most listed medications in any age group.
Commercial support: None identified.
Conclusions: Over a 23-year period in the US, office visits for psoriasis were attended mostly by white children older than 8 years with equal gender distribution. Dermatologists and pediatricians are the primary caregivers and treatment differs between these two provider groups and among children of different ages. Commercial support: None identified.
P3012 A case of benign cephalic histiocytosis with clinical and histologic features of juvenile xanthogranuloma on follow-up: Additional support for a unifying concept L. Katie Morrison, MD, University of Texas Houston, Houston, TX, United States
P3010
The etiology of histiocytic and nonhistiocytic disorders is poorly understood. NonLangerhans histiocyte (non-LCH) disorders are a group of conditions characterized by abnormal histiocytic proliferations of the non-Langerhans type. These include benign cephalic histiocytosis (BCH), juvenile xanthogranuloma (JXG), and generalized eruptive histiocytosis (GEH). A black female with a history of BCH diagnosed at 3 months of age on clinical, histologic, and immunohistochemical profiles presented for a follow-up 5 months after diagnosis. New yellowish-brown nodules were now visible, as well as the progression of the previous papular lesions into dome shaped nodules. One of these, a 1-cm nodule, was noted at the left lateral epicanthus. Also evident was the progression of some of the previous papules into multilobular nodular plaques. Her physical examination was otherwise normal. A biopsy specimen from a multilobulated plaque revealed a well-circumscribed, dense histiocytic infiltrate in the superficial dermis. Multinucleated giant cells forming nuclear wreath shapes were present, which were not visible on biopsy at 3 months of age. No foamy histiocytes were seen. Immunohistochemistry was strongly positivity for CD68 and negative for CD1a. These findings are characteristic of JXG. On ophthalmology examination there was no ocular involvement. This case clinically and histologically supports the related spectrum of BCH and JXG, which has been published from the same patient at different time points only one other time. Of note in this case are the clinical changes that prompted the additional biopsy specimen. This may indicate that cases of BCH that will subsequently demonstrate features of JXG will also have clinical changes. A multilobular nodular plaque was chosen for biopsy in this infant because of its easily accessible and cosmetically acceptable location on the posterior torso. Because there were a few distinct appearing cutaneous plaques and nodules present at the second biopsy, it is hypothesized here that a biopsy obtained from one of the larger nodular lesions would have demonstrated a more mature form of JXG with more Touton cells and foamy histiocytes. Further studies are indicated to better understand the etiology of these non-LCH disorders and their relationships. Commercial support: None identified.
FEBRUARY 2011
J AM ACAD DERMATOL
AB131