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Necrobiotic Xanthogranuloma Associated With Necrotizing Scleritis

Peyman, Amir MD*; Walsh, Noreen MD, FRCPC, FRCPath (UK)*; Green, Peter MD, FRCPC; Dorey, Michael W. MD, FRCSC; Seamone, Christopher MD, FRCSC§; Pasternak, Sylvia MD, FRCPC*

American Journal of Dermatopathology:
doi: 10.1097/DAD.0b013e318234e73c
Extraordinary Case Report
Abstract

Abstract: A 57-year-old man presented to the ophthalmology clinic with a red right eye. He denied pain, diplopia, tearing, and blurred vision. His medical history included asymptomatic annular plaques on the trunk and extremities for at least a decade. Ophthalmological examination revealed a necrotizing scleritis of the right eye. Examination of the skin demonstrated variable sized annular plaques with central atrophy, some with prominent indurated border and yellow discoloration. No periorbital lesions were present. The ocular lesion rapidly progressed and areas of scleral melting developed in the right eye, which eventually required a scleral patch graft. The left eye also developed necrotizing scleritis with areas of scleral melting. Two sets of skin biopsies were performed a few weeks apart. An initial set of skin punch biopsies revealed extensive palisading granulomatous inflammation throughout the dermis, extending into the subcutis. The accompanying perivascular mononuclear infiltrate contained the collections of plasma cells. Scattered multinucleated giant cells were noted. The possibility of necrobiosis lipoidica diabeticorum was suggested. Subsequent skin biopsies showed more prominent and extensive necrobiosis, raising the possibility of necrobiotic xanthogranuloma. Protein electrophoresis was performed, which revealed an IgG λ monoclonal protein.

Author Information

Departments of *Pathology

Dermatology, Dalhousie University, Halifax, Nova Scotia, Canada

Department of Ophthalmology, University of Alberta, Edmonton, Alberta, Canada

§Department of Ophthalmology and Visual Sciences, Dalhousie University, Halifax, Nova Scotia, Canada.

Reprints: Sylvia Pasternak, MD, FRCPC, Division of Anatomical Pathology, 5788 University Avenue, Halifax, Nova Scotia, Canada B3H 1B4 (e-mail: spastern@dal.ca).

The authors declare no conflicts of interests.

© 2012 Lippincott Williams & Wilkins, Inc.