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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Format: | Article |
Language: | English |
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Sociedade Brasileira de Dermatologia
2013-12-01
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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. |
first_indexed | 2024-12-20T15:20:19Z |
format | Article |
id | doaj.art-24b8da80806943e8bc99317b9d5f411e |
institution | Directory Open Access Journal |
issn | 0365-0596 |
language | English |
last_indexed | 2024-12-20T15:20:19Z |
publishDate | 2013-12-01 |
publisher | Sociedade Brasileira de Dermatologia |
record_format | Article |
series | Anais Brasileiros de Dermatologia |
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 |