Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report Doença de Rosai-Dorfman com manifestação cutânea extensa - Relato de caso

Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report Doença de Rosai-Dorfman com manifestação cutânea extensa - Relato de caso

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis wi...

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Bibliographic Details
Main Authors: Paula Azevedo Borges Leal, Adrilena Lopes Adriano, Marcelle Parente Breckenfeld, Igor Santos Costa, Antônio Renê Diógenes de Sousa, Heitor de Sá Gonçalves
Format: Article
Language:English
Published: Sociedade Brasileira de Dermatologia 2013-04-01
Series:Anais Brasileiros de Dermatologia
Subjects:
Emperipolese
Histiocitose
Histiocitose sinus
Histiocitose de células não Langerhans
Emperipolesis
Histiocytosis
Histiocytosis, non-langerhans-cell
Histiocytosis, sinus
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962013000200256
Description
Summary:Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.<br>A doen&#231;a de Rosai-Dorfman &#233; uma desordem benigna, autolimitada, idiop&#225;tica, decorrente da prolifera&#231;&#227;o de histi&#243;citos. Foi descrita pela primeira vez por Rosai e Dorfman em 1969. Tipicamente, a doen&#231;a se caracteriza por extensa linfadenopatia cervical, febre, gamopatia monoclonal e leucocitose com neutrofilia. Manifesta&#231;&#245;es extranodais s&#227;o encontradas em 43% dos casos, sendo a pele o s&#237;tio extranodal mais comumente afetado. No trabalho, apresentamos um caso de doen&#231;a de Rosai-Dorfman com apresenta&#231;&#227;o cut&#226;nea exuberante na coxa direita e discreto comprometimento linfonodal limitado &#224; cadeia regional.
ISSN:0365-0596
1806-4841

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