Cutaneous sinus histiocytosis and chronic uveitis

Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RD D) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately affects the lymph nodes The skin is the extranodal organ m ost frequently involved, although a few patients present with only lesions of the skin and soft tissues, without involvement of the lymph nodes (purel y cutaneous RDD). Ocular involvement is rare, and generally localized to th e eyelid. We report an unusual case of cutaneous sinus histiocytosis withou t nodal involvement and with bilateral uveitis in a girl. As far as we know , this is the first case reported in which cutaneous and ocular RDD occurre d without nodal involvement.