Abstract

IgG4 Related disease (IgG4-RD) is a novel diagnosis that was first reported in 2001. Clinical presentations are most commonly noted by systemic manifestations from secondary organ involvement with primary skin manifestation in less than 1% of the cases. We present a case of IgG4-RD that presented as proptosis in a 53 year old hispanic female. Biopsy of the left lateral rectus and lacrimal gland obtained by oculofacial plastics was noted to have a prominent lymphoid infiltrate and germinal center formation with associated zones of fibrosis. Histiocytic appearing giant cells were also present. Additional stains of IgG, IgG4 and CD138 were ordered and supported the diagnosis IgG4-RD. We present a review of the literature detailing the key pathological findings of IgG4-RD that separate this disease from other similar entities.

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