Rosai-Dorfman disease with primary cutaneous manifestations: report of two cases

Abstract

Rosai-Dorfman disease (RDD),also named as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytosis characterized by massive bilateral painless cervical lymphadenopathy and emperipolesis in tissue sections. We report the observation of 2 patients with a RDD, the first patient presented with cutaneous and lymph node involvement, while the other cutaneous only. The first patient was treated with methotrexate at a dose of 25 mg/week and oral corticosteroid 1 mg/kg/day completed by excision of the cutaneous lesions with a good evolution. Topical corticosteroids was used for the second patient with partial regression. Purely cutaneous Rosai-Dorfman disease is very rare and diagnosis is difficult without any lymphadenopathy or any distinguishing features of the skin lesion. However, a high index of suspicion by clinicians and pathologists with histopathological examination of the lesion may enable a correct diagnosis be made.