IgG4-related ophthalmic disease in association with adult-onset asthma and periocular xanthogranuloma: a case report
Details
Publication Year 2023-02,Volume 42,Issue #1,Page 98-103
Journal Title
Orbit
Publication Type
Case report
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 cells (290 per high power field) and an elevated IgG4+/IgG+ plasma cell ratio of 480%. Involvement by both disorders in the orbit and ocular adnexa of a single patient has not previously been reported in the literature, to the best of our knowledge, and suggests a possible aetiologic or pathophysiologic association.
Publisher
Taylor & Francis
Keywords
Male; Adult; Humans; Middle Aged; *Immunoglobulin G4-Related Disease/complications; Granuloma/diagnosis/drug therapy; *Xanthomatosis/complications/drug therapy/pathology; Immunoglobulin G; Eyelids/pathology; *Asthma/complications/drug therapy; Adult-onset asthma with peri-ocular xanthogranuloma; IgG4-related disease; IgG4-related ophthalmic disease; adult xanthogranulomatous disease; rituximab
Department(s)
Clinical Haematology
PubMed ID
34493168
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