A case of Rosai-Dorfman disease treated with hydroxychloroquine

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy (SMHL) is a benign histioeytic proliferative disorder of unknown etiology. It is accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. Histopamologic examinations showed characteristic 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 cases such as skin, upper respiratory tract, bone and so on. Patients with purely cutaneous Rosai-Dorfman disease are of older age at onset of the disease with a reversed male/female ratio, so cutaneous Rosai-Dorfman disease is recognized as a distinct entity from Rosai-Dorfman disease. We present a 48-year-old woman with an asymptomatic erythematous indurated plaque with yellowish papules on her left cheek and erythematous papules on her left infraorbital area, diagnosed as cutaneous Rosai-Dorfman disease at first. But 3 months later, she developed a palpable lymph node on her left submandibular area. She responded to high-dose hydroxychloroquine treatment.