TITLE:
Recurrent Bilateral Orbital Infiltration as Primary Manifestation of Rosai Dorfman Disease
AUTHORS:
Belinda Pustina, Naser Salihu, Arben Rroji, Gentian Kaloshi, Mentor Petrela
KEYWORDS:
Extranodal Rosai Dorfman, Genetic, Immunodeficiency, Histiocytosis with Massive Lymphadenopathy, Orbit, Kidney
JOURNAL NAME:
Open Journal of Ophthalmology,
Vol.7 No.3,
August
30,
2017
ABSTRACT: Rosai Dorfman disease, also known as histiocytosis with massive lymphadenopathy, is a very rare idiopathic disease. It is characterized by over production and accumulation of non Langerhans sinus histiocyte most often in lymph nodes, but may occur in other areas leading to organ damage. Based on the research, it can be caused by an infectious agent, immunodeficiency or autoimmunity and genetic causes. Extranodal manifestation is uncommon, however extranodal sites include liver, kidney, respiratory organs, orbit and eyeball. We present a case report of a 44-year-old female with recurrent bilateral orbital infiltration as first location of Rosai Dorfman disease.