Cutaneous Rosai Dorfman Disease

Abstract

A 61‐year‐old female presented with blurry vision. Questioning revealed “Flu‐like” symptoms preceding the blurry vision by two weeks. An ophthalmologist diagnosed anterior granulomatous dermatitis, which improved with prednisolone drops. One month later, an “itchy rash” and constitutional symptoms including fever, night sweats, and weight loss were reported. Physical examination revealed a viral‐type maculopapular eruption over the trunk and proximal extremities. A few weeks later, repeat examination showed progression to a diffuse, reddish‐brown, slightly edematous, papular eruption. Lymphadenopathy was not present. Laboratory data revealed a normochromic normocytic anemia, a polyclonal gammopathy, and an elevated ESR. Punch biopsies on two occasions showed a proliferation of mononucleated and multinucleated histiocytic cells with abundant pink cytoplasm. Emperipolesis was easily demonstrated. A background of plasma cells and small lymphocytes were noted. Stains for organisms were negative, and polarizable material was not present. By immunohistochemistry, histiocytes marked with S‐100 but were negative for CD1a. A diagnosis of cutaneous Rosai Dorfman was made. The cutaneous symptoms improved over six months without medical therapy. Cutaneous Rosai Dorfman disease with constitutional symptoms and laboratory evidence of systemic involvement but without lymphadenopathy has not been previously described. This unusual case suggests that a wider spectrum of disease presentation may exist.