Journal article

IgG4-related ophthalmic disease in association with adult-onset asthma and periocular xanthogranuloma: a case report

Brigitte M Papa, H Miles Prince, Alan A McNab, Penny McKelvie

ORBIT-THE INTERNATIONAL JOURNAL ON ORBITAL DISORDERS-OCULOPLASTIC AND LACRIMAL SURGERY | TAYLOR & FRANCIS INC | Published : 2021

Abstract

A 54-year-old male presented with a three-year history of bilateral upper eyelid and peri-orbital swelling and adult-onset asthma. Histopathology of a left orbital biopsy showed lymphoid follicles with foamy macrophages and Touton giant cells. Clinical, histological and radiological features were consistent with adult-onset asthma and periocular xanthogranuloma. Treatment with rituximab led to a complete clinical and radiological remission. Nine years later, his condition relapsed with a biopsy of the left orbit and lacrimal gland demonstrating features of IgG4-related disease and adult-onset asthma and periocular xanthogranuloma. Immunohistochemistry showed increased numbers of IgG4+ plasma..

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