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January 04, 2017

IgG4-Related Disease With Orbital Pseudotumors Treated With Rituximab Combined With Palpebral Surgery

Author Affiliations
  • 1Department of Dermatology, Assistance Publique-Hôpitaux de Paris (APHP), Saint-Louis Hospital, Paris VII Denis Diderot University, Paris, France
  • 2Department of Ophthalmology, Fondation Ophtalmologique A. de Rothschild, Paris, France
JAMA Dermatol. Published online January 4, 2017. doi:10.1001/jamadermatol.2016.4854

IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder featuring tumefactions and a characteristic pathological pattern. We report a case of intermittent eyelid tumefactions that led to the diagnosis of IgG4-RD 6 years after initial presentation.

A 51-year-old woman was admitted to the hospital for asymmetrical eyelid tumefactions. Six years earlier, she had had episodes of pruriginous inflammatory edema of the eyelid associated with elevation of total IgE level (1819.2 µg/L) and polyclonal hypergammaglobulinemia (1.74 g/dL). Immunological tests for antinuclear antibodies (ANAs), anti–double-stranded DNA antibodies (anti-dsDNAs), antineutrophil cytoplasmic antibodies (ANCAs), anti-Ro/SSAs, anti-La/SSBs, and complement activity produced negative results. Magnetic resonance imaging revealed periocular infiltration of the superior eyelids, including the lacrimal glands. Eyelid biopsy demonstrated a perivascular and interstitial infiltrate composed of polynuclear eosinophils and neutrophils that lacked features of lymphoma, lupus, or panniculitis. An accessory salivary gland biopsy excluded Sjögren syndrome or sarcoidosis. The diagnosis was chronic spontaneous urticaria with angioedema.

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