Cutaneous sinus histiocytosis and chronic uveitis.

Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RDD) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately affects the lymph nodes The skin is the extranodal organ most frequently involved, although a few patients present with only lesions of the skin and soft tissues, without involvement of the lymph nodes (purely cutaneous RDD). Ocular involvement is rare, and generally localized to the eyelid. We report an unusual case of cutaneous sinus histiocytosis without 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 occurred without nodal involvement.