Rosai-Dorfman disease with central nervous system involvement.

Abstract

Rosai-Dorfman disease is a rare benign disease that was first described by Rosai and Dorfman as sinus histiocytosis with massive lymphadenopathy in the 1960s.1 This disorder is documented in all age groups, but adolescents are noted to be most affected.2 The usual presentation of this disorder includes cervical lymphadenopathy, which is chronic and self-limiting. Lymphadenopathy is massive, and is associated with a raised erythrocyte sedimentation rate, anemia, pyrexia, and polyclonal hypergammaglobulinemia.2 Skin, orbital, and central nervous system (CNS) involvement are rare manifestations. Slightly more than one-third of cases present with extranodal manifestations.2 We present 3 cases of Rosai-Dorfman disease: 1 with progressive CNS involvement and 2 with disease limited to the cervical lymph nodes.