Xantogranuloma necrobiótico solitário sem paraproteinemia

Xantogranuloma necrobiótico solitário sem paraproteinemia

Alternative title Necrobiotic xanthogranuloma without paraproteinemia
Author Macedo, Danielle Mazziero Autor UNIFESP Google Scholar
Hirata, Sergio Henrique Autor UNIFESP Google Scholar
Michalany, Nílceo S. Autor UNIFESP Google Scholar
Simões, Mílvia Maria Autor UNIFESP Google Scholar
Enokihara, Silva Autor UNIFESP Google Scholar
Enokihara, Mauro Y. Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Necrobiotic xanthogranuloma is a chronic granulomatous and xantomathous disease, characterized by indurated, nontender, yellowish and erythematous nodules and plaques especially located on the periorbital region. It is commonly associated with paraproteinemia and an increased risk for hematological and lymphoproliferative malignancies. Its pathogenesis remains unclear. Alkylating agents, such as chlorambucil and melphalan may be used to treat the disease with variable success. We report a case of this rare disease in a patient with a solitary tumor and without paraproteinemia.

O xantogranuloma necrobiótico é doença crônica granulomatosa e xantomatosa, caracterizada por pápulas e placas infiltradas, eritematosas e amareladas, preferencialmente localizadas na região periorbital. É comum associar-se com paraproteinemia e risco aumentado para malignidades hematológicas e linfoproliferativas. Sua patogênese permanece desconhecida. Agentes alquilantes, como clorambucil e melfalan, podem ser utilizados no tratamento com sucesso variável. Relata-se um exemplo dessa rara doença em paciente com lesão única e sem paraproteinemia.
Keywords Granuloma
Paraproteinemias
Skin diseases, metabolic
Xanthomatosis
Dermatopatias metabólicas
Granuloma
Paraproteinemias
Xantomatose
Language Portuguese
Date 2008-06-01
Published in Anais Brasileiros de Dermatologia. Sociedade Brasileira de Dermatologia, v. 83, n. 3, p. 243-246, 2008.
ISSN 0365-0596 (Sherpa/Romeo, impact factor)
Publisher Sociedade Brasileira de Dermatologia
Extent 243-246
Origin http://dx.doi.org/10.1590/S0365-05962008000300009
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000259638100009
SciELO ID S0365-05962008000300009 (statistics in SciELO)
URI http://repositorio.unifesp.br/handle/11600/4410

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