title: Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report.
abstract: 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.
authors.affiliation: Dermatology Department, Dona Libânia Dermatology Center, Fortaleza, CE, Brazil. lealpauladl@gmail.com
authors.forename: Paula Azevedo Borges
authors.initials: PA
authors.lastname: Leal
authors.forename: Adrilena Lopes
authors.initials: AL
authors.lastname: Adriano
authors.forename: Marcelle Parente
authors.initials: MP
authors.lastname: Breckenfeld
authors.forename: Igor Santos
authors.initials: IS
authors.lastname: Costa
authors.forename: Antônio Renê Diógenes
authors.initials: AR
authors.lastname: de Sousa
authors.forename: Heitor de Sá
authors.initials: Hde S
authors.lastname: Gonçalves
mesh_terms: D001706:Biopsy; D005260:Female; D015618:Histiocytosis, Sinus / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000473:pathology*; D013848:Thigh