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January 1983

Necrobiotic Xanthogranuloma of the Eyelid

Author Affiliations

From the Oculoplastic and Orbital Services, Departments of Ophthalmology (Drs Codére and Anderson) and Dermatology (Dr Lee), University of Iowa Hospitals and Clinics, Iowa City. Dr Codère is now with the Department of Ophthalmology, McGill University, Royal Victoria Hospital, Montreal.

Arch Ophthalmol. 1983;101(1):60-63. doi:10.1001/archopht.1983.01040010062009

• Necrobiotic xanthogranuloma with paraproteinemia is characterized by multiple nodules or plaques that involve the periorbital area along with other parts of the body. A dysproteinemia due to an IgG paraprotein is associated with the condition; low serum complement, cryoglobulinemia, leukopenia, and hyperlipemia are also sometimes seen. Multiple myeloma is present in some cases. Two cases of necrobiotic xanthogranuloma with IgG monoclonal gammopathy were seen. Both initially had ocular symptoms and in the second case, the ocular manifestations have dominated the clinical picture. Histologically, these granulomas are characterized by collagen necrobiosis and by the presence of many foamy histiocytes and Touton giant cells. Because necrobiotic xanthogranuloma with monoclonal gammopathy frequently has prominent manifestations in the orbital region, may result in dysfunction of the eyelids or extraocular muscles, and is associated with potentially life-threatening systemic conditions, its recognition by the ophthalmologist is important.

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