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Treatment of necrobiotic xanthogranuloma - a systematic review.

Abstract

Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query 'necrobiotic xanthogranuloma treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG. Mainly individual case reports, small case series and retrospective studies were found. Treatment options include topical and systemic corticosteroids, thalidomide, high-dose intravenous immunoglobulin (IVIG), chlorambucil, cyclophosphamide, fludarabine, rituximab, melphalan, infliximab, interferon alpha, cladribine, hydroxychloroquine, azathioprine, methotrexate, laser therapy, radiotherapy, surgery, PUVA, plasmapheresis and extracorporeal photopheresis. Randomized controlled trials and studies on long-term outcomes after treatment were not found and are necessary to focus on in the future.

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  • Publisher Full Text
  • Authors+Show Affiliations

    ,

    Department of Dermatology, University Hospital, Jena, Germany.

    ,

    Department of Dermatology, University Hospital, Jena, Germany.

    ,

    Department of Dermatology, University Hospital, Jena, Germany.

    Department of Dermatology, University Hospital, Jena, Germany.

    Source

    MeSH

    Female
    Humans
    Male
    Necrobiotic Xanthogranuloma

    Pub Type(s)

    Journal Article
    Review
    Systematic Review

    Language

    eng

    PubMed ID

    27436448

    Citation

    Miguel, D, et al. "Treatment of Necrobiotic Xanthogranuloma - a Systematic Review." Journal of the European Academy of Dermatology and Venereology : JEADV, vol. 31, no. 2, 2017, pp. 221-235.
    Miguel D, Lukacs J, Illing T, et al. Treatment of necrobiotic xanthogranuloma - a systematic review. J Eur Acad Dermatol Venereol. 2017;31(2):221-235.
    Miguel, D., Lukacs, J., Illing, T., & Elsner, P. (2017). Treatment of necrobiotic xanthogranuloma - a systematic review. Journal of the European Academy of Dermatology and Venereology : JEADV, 31(2), pp. 221-235. doi:10.1111/jdv.13786.
    Miguel D, et al. Treatment of Necrobiotic Xanthogranuloma - a Systematic Review. J Eur Acad Dermatol Venereol. 2017;31(2):221-235. PubMed PMID: 27436448.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Treatment of necrobiotic xanthogranuloma - a systematic review. AU - Miguel,D, AU - Lukacs,J, AU - Illing,T, AU - Elsner,P, Y1 - 2016/07/19/ PY - 2015/09/09/received PY - 2016/05/02/accepted PY - 2016/7/21/pubmed PY - 2017/5/16/medline PY - 2016/7/21/entrez SP - 221 EP - 235 JF - Journal of the European Academy of Dermatology and Venereology : JEADV JO - J Eur Acad Dermatol Venereol VL - 31 IS - 2 N2 - Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query 'necrobiotic xanthogranuloma treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG. Mainly individual case reports, small case series and retrospective studies were found. Treatment options include topical and systemic corticosteroids, thalidomide, high-dose intravenous immunoglobulin (IVIG), chlorambucil, cyclophosphamide, fludarabine, rituximab, melphalan, infliximab, interferon alpha, cladribine, hydroxychloroquine, azathioprine, methotrexate, laser therapy, radiotherapy, surgery, PUVA, plasmapheresis and extracorporeal photopheresis. Randomized controlled trials and studies on long-term outcomes after treatment were not found and are necessary to focus on in the future. SN - 1468-3083 UR - https://www.unboundmedicine.com/medline/citation/27436448/Treatment_of_necrobiotic_xanthogranuloma___a_systematic_review_ L2 - https://doi.org/10.1111/jdv.13786 DB - PRIME DP - Unbound Medicine ER -