Orbital sinus histiocytosis (Rosai-Dorfman disease): a lacrimal gland involvement.

We report the clinical and histopathological features of 2 cases of orbital Rosai-Dorfman disease with lacrimal gland involvement but no lymphadenopathy or cutaneous involvement. The first case was a 7-year-old boy, who was referred to the ophthalmology department with a proptosis in the left eye. The second case involved a 57-year-old African man, who developed oedema of the right upper eyelid over a 4-month period. Ocular involvement in Rosai-Dorfman disease is rare (10%). The diagnosis is histological in combination with immunohistology and is often delicate, especially in extranodal localizations. Histopathologically, normal gland morphology was altered by fibrosis and inflammatory cells. To our knowledge, this is the first report on lacrimal involvement in incomplete, thus uncommon sinus histiocytosis with massive lymphadenopathy. Copyright 2002 S. Karger AG, Basel