Case for diagnosis. Hashimoto-Pritzker disease.

An Bras Dermatol

Pediatric Dermatology Service, Federal University of Minas Gerais, Hospital das Clínicas, Belo HorizonteMG, Brazil, Specialist in Dermatology and Pediatrics. Master in Dermatology, Federal University of Minas Gerais (UFMG). Medical Clinic of Pediatric Dermatology Service, Hospital das Clínicas, Federal University of Minas Gerais (HC-UFMG) - Belo Horizonte (MG), Brazil.

Published: September 2014

Langerhans cell histiocytosis is a rare, clinically heterogeneous disease. Since there is considerable clinical overlap among the four described variants (Hand-Schüller-Christian, eosinophilic granuloma, Letterer-Siwe and Hashimoto-Pritzker), the concept of spectral disease applies to this entity. The Hashimoto-Pritzker variant was first described in 1973. Characteristically, it is present at birth or during the first days of life, impairment is limited to the skin and prognosis is favorable with spontaneous resolution. We report a newborn male patient with Hashimoto-Pritzker disease presenting as a S100 + and CD1a + single congenital perianal lesion with rapid involution in two months.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3900360PMC
http://dx.doi.org/10.1590/abd1806-4841.20132451DOI Listing

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