Facial asymmetry with nasal and orbital involvement in a case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)

Sinus histiocytosis with massive lymphadenopathy or “Rosai-Dorfman”s syndrome is a rare benign disease of unknown etiology, usually seen in younger patients. A 12-year-old boy of the disease presented with facial asymmetry, massive lymphadenopathy, nasal and orbital manifestation is reported. Diagnosis was performed by superficial lymph node biopsy. No immunodeficiency was found. Local excision of the orbital lesion and endonasal endoscopic resection of the disease from nasal, paranasal sinuses, and nasopharynx was achieved effectively. The clinical presentation, histologic characteristics, pathogenesis and treatment of this case is discussed. This is the first reported case, to the best of our knowledge, of the disease to be associated with facial asymmetry.