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[Bilateral necrobiotic xanthogranuloma of the eyelids with associated paraproteinemia: Case report and review of literature].

Abstract

BACKGROUND

Necrobiotic xanthogranuloma is a rare disorder which predominantly affects the periorbital region and is frequently associated with paraproteinemia and lymphoproliferative diseases.

PATIENT AND METHODS

A 49-year old woman presented with bilateral, yellowish, subcutaneous lesions involving all four eyelids. These lesions have developed over the last year. Additionally, similar skin lesions developed on the trunk and the lower extremities. Two years ago, the patient had a bilateral episcleritis. An incisional biopsy was taken form the lesion. In addition, we reviewed the literature concerning similar cases.

RESULTS

Histologic examination revealed a granulomatous process consisting of histiocytes, foamy cells, multinucleated giant cells (touton- and foreign body-type) and associated with necrobiotic collagen and cholesterol clefts. General examination revealed the presence of a paraproteinemia of the IgG-typ associated with a hyperlipidemia. The patient was treated with a low dose chemotherapy which resulted in improvement of signs and symptoms. We could identify in the literature 75 similar cases. The mean age of those patients was 53 years.

CONCLUSION

The clinical and histologic findings in our patient were typical for a necrobiotic xanthogranuloma with associated paraproteinemia. The most favorable treatment response has been reported with low doses of systemic administered chemotherapeutic agents in combination with radiation therapy; however, the mortality due to underlying diseases is high.

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  • Publisher Full Text
  • Authors+Show Affiliations

    ,

    Universitäts-Augenklinik Ulm, Germany. christoph.spraul@medizin.uni-ulm.de

    ,

    Source

    MeSH

    Biopsy
    Eyelid Diseases
    Eyelids
    Female
    Granuloma, Giant Cell
    Humans
    Hyperlipidemias
    Immunoglobulin G
    Middle Aged
    Necrobiosis Lipoidica
    Paraproteinemias
    Xanthomatosis

    Pub Type(s)

    Case Reports
    English Abstract
    Journal Article
    Review

    Language

    ger

    PubMed ID

    11932812

    Citation

    Spraul, Christoph W., et al. "[Bilateral Necrobiotic Xanthogranuloma of the Eyelids With Associated Paraproteinemia: Case Report and Review of Literature]." Klinische Monatsblatter Fur Augenheilkunde, vol. 219, no. 1-2, 2002, pp. 55-8.
    Spraul CW, Wagner P, Lang GK. [Bilateral necrobiotic xanthogranuloma of the eyelids with associated paraproteinemia: Case report and review of literature]. Klin Monbl Augenheilkd. 2002;219(1-2):55-8.
    Spraul, C. W., Wagner, P., & Lang, G. K. (2002). [Bilateral necrobiotic xanthogranuloma of the eyelids with associated paraproteinemia: Case report and review of literature]. Klinische Monatsblatter Fur Augenheilkunde, 219(1-2), pp. 55-8.
    Spraul CW, Wagner P, Lang GK. [Bilateral Necrobiotic Xanthogranuloma of the Eyelids With Associated Paraproteinemia: Case Report and Review of Literature]. Klin Monbl Augenheilkd. 2002;219(1-2):55-8. PubMed PMID: 11932812.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - [Bilateral necrobiotic xanthogranuloma of the eyelids with associated paraproteinemia: Case report and review of literature]. AU - Spraul,Christoph W, AU - Wagner,P, AU - Lang,Gerhard K, PY - 2002/4/5/pubmed PY - 2002/7/19/medline PY - 2002/4/5/entrez SP - 55 EP - 8 JF - Klinische Monatsblatter fur Augenheilkunde JO - Klin Monbl Augenheilkd VL - 219 IS - 1-2 N2 - BACKGROUND: Necrobiotic xanthogranuloma is a rare disorder which predominantly affects the periorbital region and is frequently associated with paraproteinemia and lymphoproliferative diseases. PATIENT AND METHODS: A 49-year old woman presented with bilateral, yellowish, subcutaneous lesions involving all four eyelids. These lesions have developed over the last year. Additionally, similar skin lesions developed on the trunk and the lower extremities. Two years ago, the patient had a bilateral episcleritis. An incisional biopsy was taken form the lesion. In addition, we reviewed the literature concerning similar cases. RESULTS: Histologic examination revealed a granulomatous process consisting of histiocytes, foamy cells, multinucleated giant cells (touton- and foreign body-type) and associated with necrobiotic collagen and cholesterol clefts. General examination revealed the presence of a paraproteinemia of the IgG-typ associated with a hyperlipidemia. The patient was treated with a low dose chemotherapy which resulted in improvement of signs and symptoms. We could identify in the literature 75 similar cases. The mean age of those patients was 53 years. CONCLUSION: The clinical and histologic findings in our patient were typical for a necrobiotic xanthogranuloma with associated paraproteinemia. The most favorable treatment response has been reported with low doses of systemic administered chemotherapeutic agents in combination with radiation therapy; however, the mortality due to underlying diseases is high. SN - 0023-2165 UR - https://www.unboundmedicine.com/medline/citation/11932812/[Bilateral_necrobiotic_xanthogranuloma_of_the_eyelids_with_associated_paraproteinemia:_Case_report_and_review_of_literature]_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2000-23502 DB - PRIME DP - Unbound Medicine ER -