A Case of Cutaneous Rosai-Dorfman Disease Manifesting as Multiple Tumors

Bae, You In; Lee, Jee Bum; Kim, Seong Jin; Lee, Seung Chul; Won, Young Ho; Yun, Sook Jung

Abstract

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is an idiopathic, benign, self-limited, histiocytic proliferative disease confined mainly to cervical lymph nodes. The disease is usually accompanied by massive bilateral lymphadenopathy, fever, elevated erythrocyte sedimentation rate, leukocytosis with neutrophilia, and polyclonal hypergammaglobulinemia. Although cutaneous manifestations are observed in 10% of patients, pure cutaneous RDD without any nodal or systemic involvement is very rare. We report a case of pure cutaneous RDD occurring in a 37-year-old man with asymptomatic erythemaotus to yellowish multiple widespread nodules and tumors on the face and trunk. Histopathologic examination characteristically showed a diffuse infiltrate composed of large histiocytes exhibiting emperiopolesis and mixed inflammatory cells including many plasma cells. Immunohistochemically, these histiocytes were positive for S-100 protein and CD68, but negative for CD1a. During 2 years of follow-up, the patient showed neither nodal nor systemic involvement of the disease and the skin lesions gradually disappeared. The final diagnosis was made by typical cutaneous manifestations without nodal or extranodal involvement and histopathological findings compatible with RDD.