Case for diagnosis. Hashimoto-Pritzker disease.
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.
Hospital das Clínicas, Federal University of Minas Gerais, Belo HorizonteMG, Brazil, Medical Resident. Dermatology Unit, Hospital das Clínicas, Federal University of Minas Gerais (HC-UFMG) - Belo Horizonte (MG), Brazil.
Virginia Vinha
VV
Zanuncio
Hospital das Clínicas, Federal University of Minas Gerais, Belo HorizonteMG, Brazil, Medical Resident. Dermatology Unit, Hospital das Clínicas, Federal University of Minas Gerais (HC-UFMG) - Belo Horizonte (MG), Brazil.
Luciana Rabelo de
LR
Carvalho
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.
Antônio Carlos Martins
AC
Guedes
School of Medicine, Federal University of Minas Gerais, Belo HorizonteMG, Brazil, MD, PHD. Associate Professor of Dermatology, Federal University of Minas Gerais School of Medicine (UFMG) - Belo Horizonte (MG), Brazil.
Cláudia Márcia Resende
CM
Silva
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.
Bernardo
B
Gontijo
D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012871:Skin Diseases / Q000473:pathology*