Recurrent Bilateral Orbital Infiltration as Primary Manifestation of Rosai Dorfman Disease

• 作者: Arben Rroji Belinda Pustina Gentian Kaloshi Mentor Petrela Naser Salihu
• 来源期刊: 眼科学期刊(英文)
• 学科: 医学
• 关键词: EXTRANODAL Rosai Dorfman Genetic IMMUNODEFICIENCY HISTIOCYTOSIS with Massive LYMPHADENOPATHY Orbit Kidney
• 年，卷（期）: 2017,（3）
• 页码范围: 225-229
• 页数: 5页
• 分类号: R73

摘要

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.