Cutaneous Rosai-Dorfman disease: A separate clinical entity

Abstract

Rosai-Dorfman disease is a benign, self-limited non-Langerhans cell histiocytosis of unknown etiology. Its most common clinical feature is extraordinary massive painless cervical lymphadenopathy. This entity may be limited to the lymph nodes; however, more than 40% of patients have extranodal involvement, with the skin being the most frequently affected site. Cutaneous disease without the presence of lymphadenopathy is extremely rare but has been reported. We report the intriguing case of exclusively cutaneous Rosai-Dorfman disease in an 80-year-old African-American woman.