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. T...

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Main Authors: Virginia Vinha Zanuncio, Luciana Rabelo de Carvalho, Antônio Carlos Martins Guedes, Cláudia Márcia Resende Silva, Bernardo Gontijo
Format: Article
Language:English
Published: Sociedade Brasileira de Dermatologia 2013-12-01
Series:Anais Brasileiros de Dermatologia
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962013000601001&tlng=en
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author Virginia Vinha Zanuncio
Luciana Rabelo de Carvalho
Antônio Carlos Martins Guedes
Cláudia Márcia Resende Silva
Bernardo Gontijo
author_facet Virginia Vinha Zanuncio
Luciana Rabelo de Carvalho
Antônio Carlos Martins Guedes
Cláudia Márcia Resende Silva
Bernardo Gontijo
author_sort Virginia Vinha Zanuncio
collection DOAJ
description 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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spelling doaj.art-24b8da80806943e8bc99317b9d5f411e2022-12-21T19:36:04ZengSociedade Brasileira de DermatologiaAnais Brasileiros de Dermatologia0365-05962013-12-018861001100310.1590/abd1806-4841.20132451Case for diagnosisVirginia Vinha ZanuncioLuciana Rabelo de CarvalhoAntônio Carlos Martins GuedesCláudia Márcia Resende SilvaBernardo GontijoLangerhans 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962013000601001&tlng=enHistiocytosis, Langerhans-cellImmunohistochemistryPrognosis
spellingShingle Virginia Vinha Zanuncio
Luciana Rabelo de Carvalho
Antônio Carlos Martins Guedes
Cláudia Márcia Resende Silva
Bernardo Gontijo
Case for diagnosis
Anais Brasileiros de Dermatologia
Histiocytosis, Langerhans-cell
Immunohistochemistry
Prognosis
title Case for diagnosis
title_full Case for diagnosis
title_fullStr Case for diagnosis
title_full_unstemmed Case for diagnosis
title_short Case for diagnosis
title_sort case for diagnosis
topic Histiocytosis, Langerhans-cell
Immunohistochemistry
Prognosis
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962013000601001&tlng=en
work_keys_str_mv AT virginiavinhazanuncio casefordiagnosis
AT lucianarabelodecarvalho casefordiagnosis
AT antoniocarlosmartinsguedes casefordiagnosis
AT claudiamarciaresendesilva casefordiagnosis
AT bernardogontijo casefordiagnosis