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"The term "sinus histiocytosis with massive lyrnphadenopathy," introduced in 1969 by Rosai and Dorfman, refers to a newly recognized disease entity characterized by painless cervical lymphadenopathy, fever, leukocytosis, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. The typical course is one of insidious onset, protracted duration of the active disease state, and eventual spontaneous remission, occasionally with subsequent recurrences. Lymph nodes other than those in the cervical area may be involved, and extranodal involvement can occur (such as in the orbit, skin, or respiratory tract) Clinically, this entity may closely simulate malignant lymphoma or nonneoplastic conditions with lymphadenopathy. We present a case in which this disease process involved the perineum."
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