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.