Case for diagnosis*

Virginia Vinha Zanuncio, Luciana Rabelo de Carvalho, Antônio Carlos Martins Guedes, Cláudia Márcia Resende Silva, Bernardo Gontijo

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.