Atypical cases of sinus histiocytosis (Rosai-Dorfman disease) with ophthalmological manifestations.

Sinus histiocytosis with massive lymphadenopathy is a non-neoplastic disease of unknown cause observed mainly in children with markedly enlarged cervical or other lymph nodes. We have reported two very atypical cases with extranodal manifestations that required ophthalmological consultation. Neither patient had significant lymphadenopathy. One patient, who had tumors of all four eyelids as well as lesions of the nose and larynx, was 68 years old when first examined, and 70 at the time of his last surgical procedure. The other patient, a 13-month-old child, had a unique corneoscleral lesion as his only clinical manifestation. The excised tissues obtained from the eyelids and larynx in case 1 and from the corneoscleral lesion in case 2 revealed characteristic histopathologic features of sinus histiocytosis (Rosai-Dorfman disease), including strongly positive immunoperoxidase staining for S-100 protein in the cytoplasm of most of the histiocytes.