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[Necrobiotic xanthogranuloma with scleroatrophic lichen associated with paraproteinemia].

Abstract

Necrobiotic xanthogranuloma (Xn) with paraproteinemia is a histiocytoxanthomatosis (non-X histiocytosis) that affects the dermis and subcutaneous tissue of the face and less frequently the trunk and limbs. We present the case of a 58-year-old woman with a previous background of IgG (lambda) paraproteinemia and multiple autoimmune diseases, that associate clinically and histologically typical lesions of Xn on face, neck and limbs and of lichen sclerosus et atrophius (LEA) on skin and mucosae. The treatments performed were ineffective, the Xn lesions followed a chronic and progressive course with increased number, size and ulceration of them. The paraproteinemia has remained stable since it was diagnosed eight years ago. We have not found the association of Xn with paraproteinemia and SAL described in the literature. We review the characteristics of this rare disease and its possible pathogenic mechanisms.

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  • Authors+Show Affiliations

    ,

    Servicio de Dermatología, Complejo Hospitalario Xeral-Calde de Lugo, San Roque 25, 27002 Lugo, Spain. yojuarezca@yahoo.es

    , , ,

    Source

    Actas dermo-sifiliograficas 97:9 2006 Nov pg 603-8

    MeSH

    Female
    Glucocorticoids
    Granuloma
    Humans
    Lichen Sclerosus et Atrophicus
    Middle Aged
    Necrobiotic Disorders
    Paraproteinemias
    Skin
    Treatment Outcome
    Xanthomatosis

    Pub Type(s)

    Case Reports
    English Abstract
    Journal Article

    Language

    spa

    PubMed ID

    17173768

    Citation

    Juárez, Y, et al. "[Necrobiotic Xanthogranuloma With Scleroatrophic Lichen Associated With Paraproteinemia]." Actas Dermo-sifiliograficas, vol. 97, no. 9, 2006, pp. 603-8.
    Juárez Y, España S, Lueiro M, et al. [Necrobiotic xanthogranuloma with scleroatrophic lichen associated with paraproteinemia]. Actas Dermosifiliogr. 2006;97(9):603-8.
    Juárez, Y., España, S., Lueiro, M., Fernández-Díaz, M. L., & Bal, F. (2006). [Necrobiotic xanthogranuloma with scleroatrophic lichen associated with paraproteinemia]. Actas Dermo-sifiliograficas, 97(9), pp. 603-8.
    Juárez Y, et al. [Necrobiotic Xanthogranuloma With Scleroatrophic Lichen Associated With Paraproteinemia]. Actas Dermosifiliogr. 2006;97(9):603-8. PubMed PMID: 17173768.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - [Necrobiotic xanthogranuloma with scleroatrophic lichen associated with paraproteinemia]. AU - Juárez,Y, AU - España,S, AU - Lueiro,M, AU - Fernández-Díaz,M L, AU - Bal,F, PY - 2006/12/19/pubmed PY - 2007/2/28/medline PY - 2006/12/19/entrez SP - 603 EP - 8 JF - Actas dermo-sifiliograficas JO - Actas Dermosifiliogr VL - 97 IS - 9 N2 - Necrobiotic xanthogranuloma (Xn) with paraproteinemia is a histiocytoxanthomatosis (non-X histiocytosis) that affects the dermis and subcutaneous tissue of the face and less frequently the trunk and limbs. We present the case of a 58-year-old woman with a previous background of IgG (lambda) paraproteinemia and multiple autoimmune diseases, that associate clinically and histologically typical lesions of Xn on face, neck and limbs and of lichen sclerosus et atrophius (LEA) on skin and mucosae. The treatments performed were ineffective, the Xn lesions followed a chronic and progressive course with increased number, size and ulceration of them. The paraproteinemia has remained stable since it was diagnosed eight years ago. We have not found the association of Xn with paraproteinemia and SAL described in the literature. We review the characteristics of this rare disease and its possible pathogenic mechanisms. SN - 0001-7310 UR - https://www.unboundmedicine.com/medline/citation/17173768/[Necrobiotic_xanthogranuloma_with_scleroatrophic_lichen_associated_with_paraproteinemia]_ L2 - http://www.elsevier.es/en/linksolver/pdf/pii/13095279 DB - PRIME DP - Unbound Medicine ER -