Track

Case Reports

Abstract

Rosai-Dorfman disease (RDD) is a rare benign proliferation of histiocytes that typically occurs systemically with fever, massive, painless, bilateral cervical lymphadenopathy, and involvement of extranodal sites, including the skin, oral and nasal cavities, and periorbital tissue. In the systemic form of disease, the skin is involved in about 10% of patients.  However, RDD can present solely with cutaneous lesions without systemic findings, an extremely rare variant known as cutaneous Rosai-Dorfman disease (CRDD). CRDD typically presents as single or multiple red-brown or xanthomatous macules, papules, nodules, or plaques that are usually on the head and neck. Herein, we describe a diagnostically challenging case of CRDD on the nasal dorsum of a 64-year-old female. Histopathological analysis of a cutaneous punch biopsy revealed a dense multinodular hematolymphoid proliferation containing plasma cells, large polygonal histiocytes with abundant feathery cytoplasm and emperipolesis, and storiform fibrosis involving the nasal skeletal muscle, subcutis, and dermis. Immunohistochemistry revealed the enlarged dermal histiocytes expressed S100, OCT2, and CD68; while CD1a expression was absent. In totality, we present a unique and extraordinary case of cutaneous Rosai-Dorfman Disease, highlighting the histopathologic pearls and clinical features of this uncommon cutaneous disease.