Rhinoscleroma and Rosai–Dorfman disease have been reported to coexist in the same patient at different sites. Rosai–Dorfman disease may have an aetiological relationship with rhinoscleroma, although this has not yet been proved.

A case of a 42-year-old woman with recurrent nasal masses is presented, with histopathological proof of both conditions coexisting in the same nasal lesion.

The aetiopathology, clinical features and treatment of both diseases are discussed and a literature survey is reported. Histologically, the presence of Mikulicz cells with entrapped, rod-like, Gram-negative bacilli and Russell bodies suggests rhinoscleroma. Emperipolesis and S-100-positive histiocytes confirm the diagnosis of Rosai-Dorfman disease. The presence of both in the same slides from affected tissues has never been demonstrated before. In the light of this evidence, the author believes that rhinoscleroma must be considered in the aetiology of Rosai–Dorfman disease.

Rosai–Dorfman disease is a rare, idiopathic, histiocytic proliferative disorder with a distinctive microscopic appearance, which was formerly thought to be a disease process limited to lymph nodes. However, extranodal involvement has been documented in less than half of the reported patients, but rarely without associated lymphadenopathy.

We report the case of a 43-year-old Senegalese woman who presented with a polypoid, intranasal mass caused by Rosai–Dorfman disease. A diagnosis of a granulomatous process, including rhinoscleroma, was initially discussed. The correct diagnosis was made histologically by demonstrating aggregates of histiocytes with large amounts of cytoplasm, emperipolesis and protein S100 antigen expression. Despite using ancillary methods (molecular biology and electron microscopy), we failed to demonstrate any associated pathogen.

Diagnosis of Rosai–Dorfman disease can be very difficult, in particular in adults from Africa with pure, isolated, intranasal localisation, in whom clinical and radiological features may mimic other infectious or neoplastic disorders. The diagnosis is made based on the histological presence of large histiocytes with lymphophagocytosis. Moreover, immunohistochemical analysis of these histiocytes using anti-protein S100 antibody shows strong positivity.