Case for diagnosis

Langerhans cell histiocytosis is a rare, clinically heterogeneous desease. 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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Bibliographic Details
Main Authors: Zanuncio,Virginia Vinha, Carvalho,Luciana Rabelo de, Guedes,Antônio Carlos Martins, Silva,Cláudia Márcia Resende, Gontijo,Bernardo
Format: Digital revista
Language:English
Published: Sociedade Brasileira de Dermatologia 2013
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962013000601001
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