Erdheim Chester Disease

A 37-year-old male presented to clinic with bilateral conjunctival lesions for 7 months. Patient has a history of lytic bone lesions, 50-pound weight loss, and fevers. Examination revealed 20/20 vision in both eyes, mobile, discrete conjunctival lesions in bilateral bulbar conjunctiva, and right cervical adenopathy. Conjunctival biopsy revealed histiocytosis and flow cytometry was negative for lymphoma. Previous bone marrow biopsy showed atypical histiocytic infiltrate without abnormal T or B cells. Inguinal lymph node biopsy showed foamy macrophages suggestive of Erdheim-Chester Disease. Patient underwent systemic work up and was treated with high dose steroids by Hematology-Oncology.

Presentation Date: 08/20/2020

Issue Date: 02/05/2021