Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report*

نویسندگان

  • Paula Azevedo Borges Leal
  • Adrilena Lopes Adriano
  • Marcelle Parente Breckenfeld
  • Igor Santos Costa
  • Antônio Renê Diógenes de Sousa
  • Heitor de Sá Gonçalves
چکیده

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.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Rosai-Dorfman disease successfully treated with thalidomide

ESR: erythrocyte sedimentation rate INTRODUCTION Rosai-Dorfman disease, also called sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic condition of unknown etiology first described in 1969. Primary cutaneous manifestation without systemic involvement of Rosai-Dorfman disease is even more uncommon. Here we present a case of primary cutaneous manifestation of Rosai-Dorfman di...

متن کامل

Rosai-Dorfman Disease: A Case Report and Literature Review

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disorder that typically manifests as lymphadenopathy and systemic symptoms whose etiology remains poorly elucidated. The diagnosis is based on immunohistochemistry. Its treatment is poorly defined but the prognosis is usually favorable. Here we report a 14 year old boy who presented with massive bilateral cervi...

متن کامل

Rosai-Dorfman disease with primary cutaneous manifestations--a case report.

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a benign systemic proliferative disorder of histiocytes resembling the sinus histiocytes of lymph nodes. The typical clinical features of this disease include bilateral painless lymphadenopathy, fever, and polyclonal hyperglobulinaemia. The condition may present with extranodal involvement in 43% of cases, and cutaneous...

متن کامل

A case of isolated cutaneous Rosai-Dorfman-Syndrome.

UNLABELLED Rosai-Dorfman Syndrome (RDD) is a rare, benign, self-limiting disorder which is characterized by the non-malignant proliferation of distinctive histiocytic cell within lymphatic system. RDD has been described as a dynamic entity in the spectrum of histiocytosis with non-Langerhans cell histiocytosis at one end and Langerhans cell histiocytosis at the other. The exact etiology of this...

متن کامل

Cutaneous rosai dorfman disease: A case report and literature review.

This study reports a clinical case of cutaneous Rosai-Dorfman disease, its manifestation, and its evolution in 10 years, thus contributing to the limited bibliography about it and which review is also an important aim of this paper. This clinical study included a histopathological and immunohistochemical review of the lesions. Since this is a skin disease, not a life-threatening condition, and ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 88  شماره 

صفحات  -

تاریخ انتشار 2013