Cutaneous Rosai-Dorfman Disease - A Review of 17 Cases

Cutaneous Rosai-Dorfman disease (RDD) is a rare entity of unknown etiology. In Taiwan, only scattered cases had been reported. In this retrospective study, we review all patients with pathology- proven RDD diagnosed at Chang - Gung Memorial Hospital from 1995 to 2002, and report the demography, clinical manifestations, pathological characteristics, treatment modalities and prognosis of this entity. The presented series consists of 17 patients of cutaneous RDD, including ten female and seven males, aged between 23 and 75 years. The clinical appearance was variable, but most commonly involved a central noduloplaque with satellite papules. Multifocal involvement only occurred in three patients. Concurrent involvement of uvea or vocal cord occurred in two patients. No involvement of lymph node or internal organ was noticed among these patients during the follow-up periods. The most prominent histological feature was a florid and mixed inflammatory infiltration. Emperipolesis, a characteristic finding of nodal RDD, was usually only focally presented. Positivity for S - 100 protein assisted to confirm the diagnosis. The most effective treatment was surgical excision of solitary or symptomatic lesions. High dose thalidomide (300 mg per day) may be used to control the extensive cutaneous diseases, though its efficacy should be further evaluated. Three patients experienced spontaneous remission one to two years after diagnosis. (Dermatol Sinica 22 : 114-123,2004)