[Bilateral necrobiotic xanthogranuloma of the eyelids with associated paraproteinemia: Case report and review of literature]. / Bilaterales nekrobiotisches Xanthogranulom der Lider mit Paraproteinämie: Fallbericht sowie Literaturübersicht.
Klin Monbl Augenheilkd
; 219(1-2): 55-8, 2002.
Article
em De
| MEDLINE
| ID: mdl-11932812
ABSTRACT
BACKGROUND:
Necrobiotic xanthogranuloma is a rare disorder which predominantly affects the periorbital region and is frequently associated with paraproteinemia and lymphoproliferative diseases. PATIENT ANDMETHODS:
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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Base de dados:
MEDLINE
Assunto principal:
Paraproteinemias
/
Granuloma de Células Gigantes
/
Xantomatose
/
Doenças Palpebrais
/
Necrobiose Lipoídica
Idioma:
De
Ano de publicação:
2002
Tipo de documento:
Article