A Case of Cutaneous Rosai-Dofman Disease Treated with Isotretinoin and Pulsed Dye Laser / 대한피부과학회지

ABSTRACT

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SMHL) is a benign histiocytic proliferative disorder of unknown etiology. The disease is usually accompanied by massive bilateral lymphadenopathy, fever, elevated erythrocyte sedimentation rate, leukocytosis with neutrophilia, and polyclonal hypergammaglobulinemia. Histopathologic examinations showed characteristically large histiocytes exhibiting emperipolesis. On immunohistochemical stains, histiocytes are positive for CD68 and S-100 protein, but negative for CD1a. The lymph node involvement is typical, but it may also involve other systemic organs in one third of the cases such as skin, upper respiratory tract, bones and so on. Patients with purely cutaneous Rosai-Dorfman diseases are of older age at onset of the disease with a reversed male/female ratio, thus, cutaneous Rosai-Dorfman disease is recognized as a distinct entity from the Rosai-Dorfman disease. Herein, we present a 50-year-old man with erythematous papules and indurated plaques on both cheeks, diagnosed as cutaneous Rosai-Dorfman disease. The lesions were treated with isotretinoin 10 mg bid for 9 months with pulsed dye laser.