Primary Cutaneous Rosai-Dorfman Disease Presenting as an Infiltrative Dorsal Nasal Mass With Deep Skeletal Muscle Involvement.
Luke Hamilton, Julien C Bourgeois, Katherine Johnson et al.
✦ AI-curated · Sources linked
Case in brief
A 64-year-old woman had an 18-month history of a progressively enlarging, pruritic mass involving the nasal dorsum and both sidewalls. It had not responded to multiple therapies and was repeatedly diagnosed as granulomatous rosacea. Deep biopsy showed a dense lymphoplasmacytic infiltrate with storiform fibrosis extending through the dermis and subcutis into skeletal muscle. Large histiocytes showed emperipolesis and expressed S100, OCT2, and CD68, with negative CD1a staining. Ancillary studies excluded infection and hematolymphoid malignancy, supporting primary cutaneous Rosai-Dorfman disease.
What made this case unusual
The infiltrative nasal lesion extended into skeletal muscle, an unusual and histopathologically confirmed feature of cutaneous Rosai-Dorfman disease.
Diagnostic / clinical pearl
A persistent, treatment-refractory nasal mass diagnosed as granulomatous rosacea may warrant deep biopsy; identifying emperipolesis and the histiocytic immunophenotype can reveal cutaneous Rosai-Dorfman disease.