分子与遗传医学

Recurrent Bilateral Orbital Infiltration as Primary Manifestation of Rosai Dorfman Disease

Abstract

Pustina B, Salihu N, Rroji A, Kaloshi G and Petrela M

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.