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Necrobiotic xanthogranuloma with paraproteinemia: an evolving presentation.
Cutis 1997; 59(6):333-6C

Abstract

Necrobiotic xanthogranuloma with paraproteinemia is a progressive and destructive process that is often confused both clinically and histologically with other granulomatous and xanthomatous entities. It was first described by Kossard and Winkelmann in 1980. Prior to this, the entity was reported under a variety of names such as atypical multicentric reticulohistiocytosis with paraproteinemia, atypical xanthoma disseminatum, and atypical necrobiosis lipoidica. A 69-year-old woman experienced slightly pruritic and painful papules, plaques, and nodules. Initial biopsy specimens showed a granulomatous process consistent with granuloma annulare. Later biopsy specimens demonstrated histologic changes indicative of necrobiosis lipoidica. The most recent histologic findings are those of necrobiotic xanthogranuloma, with results of laboratory studies revealing a coexistent IgG kappa paraproteinemia. Patients with necrobiotic xanthogranuloma who demonstrate a benign monoclonal proteinemia and are evaluated for several years show a 9 to 11 percent risk of myeloma, amyloidosis, or macroglobulinemia. Multiple treatment regimens have been attempted, none of which are curative. We propose that this entity may be part of an evolutionary process that may start as a granulomatous entity and culminate in a xanthogranulomatous process with an accompanying paraproteinemia.

Authors+Show Affiliations

Department of Dermatology, Wilford Hall Medical Center/PSMD, Lackland Air Force Base, Texas 78236-5300, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

9218893

Citation

Johnston, K A., et al. "Necrobiotic Xanthogranuloma With Paraproteinemia: an Evolving Presentation." Cutis, vol. 59, no. 6, 1997, pp. 333-6.
Johnston KA, Grimwood RE, Meffert JJ, et al. Necrobiotic xanthogranuloma with paraproteinemia: an evolving presentation. Cutis. 1997;59(6):333-6.
Johnston, K. A., Grimwood, R. E., Meffert, J. J., & Deering, K. C. (1997). Necrobiotic xanthogranuloma with paraproteinemia: an evolving presentation. Cutis, 59(6), pp. 333-6.
Johnston KA, et al. Necrobiotic Xanthogranuloma With Paraproteinemia: an Evolving Presentation. Cutis. 1997;59(6):333-6. PubMed PMID: 9218893.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Necrobiotic xanthogranuloma with paraproteinemia: an evolving presentation. AU - Johnston,K A, AU - Grimwood,R E, AU - Meffert,J J, AU - Deering,K C, PY - 1997/6/1/pubmed PY - 1997/6/1/medline PY - 1997/6/1/entrez SP - 333 EP - 6 JF - Cutis JO - Cutis VL - 59 IS - 6 N2 - Necrobiotic xanthogranuloma with paraproteinemia is a progressive and destructive process that is often confused both clinically and histologically with other granulomatous and xanthomatous entities. It was first described by Kossard and Winkelmann in 1980. Prior to this, the entity was reported under a variety of names such as atypical multicentric reticulohistiocytosis with paraproteinemia, atypical xanthoma disseminatum, and atypical necrobiosis lipoidica. A 69-year-old woman experienced slightly pruritic and painful papules, plaques, and nodules. Initial biopsy specimens showed a granulomatous process consistent with granuloma annulare. Later biopsy specimens demonstrated histologic changes indicative of necrobiosis lipoidica. The most recent histologic findings are those of necrobiotic xanthogranuloma, with results of laboratory studies revealing a coexistent IgG kappa paraproteinemia. Patients with necrobiotic xanthogranuloma who demonstrate a benign monoclonal proteinemia and are evaluated for several years show a 9 to 11 percent risk of myeloma, amyloidosis, or macroglobulinemia. Multiple treatment regimens have been attempted, none of which are curative. We propose that this entity may be part of an evolutionary process that may start as a granulomatous entity and culminate in a xanthogranulomatous process with an accompanying paraproteinemia. SN - 0011-4162 UR - https://www.unboundmedicine.com/medline/citation/9218893/Necrobiotic_xanthogranuloma_with_paraproteinemia:_an_evolving_presentation_ L2 - http://www.diseaseinfosearch.org/result/7560 DB - PRIME DP - Unbound Medicine ER -