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[播散性结节性原发性局限性皮肤淀粉样变性]

[Disseminated nodular primary localized cutaneous amyloidosis].

作者信息

Feito-Rodríguez M, García-Macarrón J, Pagán-Muñoz B, Mariño-Enríquez A, Vidaurrázaga-Díaz Y Arcaya C, Díaz-Díaz R M, Casado-Jiménez M

机构信息

Servicio de Dermatología, Hospital Universitario La Paz, Madrid, España.

出版信息

Actas Dermosifiliogr. 2008 Oct;99(8):648-52.

Abstract

Amyloid is a proteinaceous material that is deposited in the tissues in a large variety of clinical contexts; in the skin it can be found with or without concomitant systemic disease. Primary localized cutaneous amyloidosis encompasses those amyloidoses restricted to the skin without involvement of other systems. The most common forms within this group are macular and lichen amyloidosis. Nodular amyloidosis is extremely rare, and there are notable differences in clinical presentation, prognosis, histology, and pathogenesis between this entity and the macular and lichenoid variants. We report a new case of nodular primary localized cutaneous amyloidosis with disseminated plaques and nodules in which no systemic disease developed in the 3 years following the appearance of the lesions.

摘要

淀粉样蛋白是一种蛋白质物质,在多种临床情况下沉积于组织中;在皮肤中,无论有无伴随的全身性疾病均可发现。原发性局限性皮肤淀粉样变包括那些局限于皮肤而无其他系统受累的淀粉样变。该组中最常见的形式是斑状和苔藓样淀粉样变。结节状淀粉样变极为罕见,该类型与斑状和苔藓样变体在临床表现、预后、组织学及发病机制方面存在显著差异。我们报告一例新的结节状原发性局限性皮肤淀粉样变病例,伴有散在斑块和结节,病变出现后3年未发生全身性疾病。

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