Extranodal Rosai–Dorfman disease in an adolescent patient
Arwa Albalawi, Dana Alsarhani, Salem Balhamar, Ali Alasmari, Mohammed AlrajehRosai–Dorfman disease (RDD) is a rare histiocytic disorder, typically presenting with bilateral cervical lymphadenopathy. Extranodal RDD, especially isolated orbital involvement, is rare and considered a diagnostic challenge. A 16-year-old male with recurrent nasal masses, excised multiple times, presented with 7 months of progressive right periorbital swelling and pain. Ophthalmic exam revealed proptosis, ptosis, and mild restriction of extraocular movements. Orbital imaging showed an enlarging right orbital mass extending into the post-septal space, with no systemic symptoms or lymphadenopathy. A biopsy revealed fibroconnective tissue infiltrated by histiocytes, lymphocytes, and plasma cells. Immunohistochemical staining for S100, CD68, and CD163 confirmed RDD.