Abstract

Rosai-Dorfman disease (RDD), also referred to as sinus histiocytosis with massive lymphadenopathy (SHML), is a rareproliferative disorder of phagocytic histiocytes of unknown etiology. Systemic RDD most typically presents as painless cervical lymphadenopathy with constitutional symptoms. Extranodal involvement by RDD has been reported in nearly half of all cases, including skin, head/neck and upper respiratory tract. Genitourinary involvement has rarely been described. We report a case of a 6-year-old girl who presented with a large firm vulvar mass with greenish discoloration of the overlying skin. Partial surgical excision was performed at the request of the patient's parents, and biopsy results were consistent with extranodal RDD.We present this case in order to increase awareness of the diagnosis and variable presentations of Rosai-Dorfman disease.

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