获得性皮肤松弛症伴重链沉积病

Acquired cutis laxa associated with heavy chain deposition disease.

作者信息

Harrington Cynthia R, Beswick Tracy C, Susa Joseph S, Pandya Amit G

机构信息

Department of Dermatology, The University of Texas Southwestern Medical Center, Dallas, Texas 75390-9190, USA.

出版信息

J Am Acad Dermatol. 2008 Nov;59(5 Suppl):S99-101. doi: 10.1016/j.jaad.2008.05.007.

DOI:10.1016/j.jaad.2008.05.007

PMID:19119136

Abstract

Acquired cutis laxa (generalized acquired elastolysis) is a condition of unknown etiology characterized by a degeneration of elastic fibers in the skin, resulting in laxity with reduced elastic recoil. We describe a patient with acquired cutis laxa associated with an underlying heavy chain deposition disease. Direct immunofluorescence testing of lesional skin demonstrated immunoglobulin G deposition on elastic fibers, suggesting that in some cases, cutis laxa may have an immune-mediated etiopathogenesis.

摘要

获得性皮肤松弛症（全身性获得性弹性组织离解）是一种病因不明的病症，其特征为皮肤弹性纤维变性，导致皮肤松弛且弹性回缩减弱。我们描述了一名患有获得性皮肤松弛症且伴有潜在重链沉积病的患者。对皮损皮肤进行直接免疫荧光检测显示免疫球蛋白G沉积于弹性纤维上，这表明在某些情况下，皮肤松弛症可能具有免疫介导的发病机制。