Tan Stephen, Pon Kucy, Bargman Joanne, Ghazarian Danny
University Hospital, Indianapolis, Indiana 46202, USA,
J Cutan Med Surg. 2003 Sep-Oct;7(5):390-4. doi: 10.1007/s10227-002-0128-z. Epub 2003 Sep 24.
Cutis laxa is a heterogeneous group of inherited and acquired disorders characterized clinically by loose skin and histologically by altered elastic tissue. Heavy chain deposition disease is a very rare monoclonal immunoglobulin disorder, distinct from multiple myeloma, in which there is production and deposition of defective immunoglobulin heavy chains without light chain deposition.
We describe a case of acquired cutis laxa associated with heavy chain deposition disease.
A 50-year-old male presented with acute renal failure, IgG4 heavy chain deposition in the kidneys, and no evidence of multiple myeloma. Four years later, he developed generalized acquired cutis laxa, emphysema, and a peripheral polyneuropathy. On pathology, there was destruction of elastic fibers within the dermis.
This case describes a previously unreported association between acquired cutis laxa and heavy chain deposition disease.
皮肤松弛症是一组遗传性和获得性疾病的异质性群体，临床特征为皮肤松弛，组织学特征为弹性组织改变。重链沉积病是一种非常罕见的单克隆免疫球蛋白疾病，与多发性骨髓瘤不同，其特征是产生并沉积有缺陷的免疫球蛋白重链而无轻链沉积。
我们描述一例与重链沉积病相关的获得性皮肤松弛症病例。
一名50岁男性出现急性肾衰竭，肾脏有IgG4重链沉积，且无多发性骨髓瘤证据。四年后，他出现全身性获得性皮肤松弛症、肺气肿和周围性多发性神经病。病理检查显示真皮内弹性纤维破坏。
本病例描述了获得性皮肤松弛症与重链沉积病之间一种此前未报道的关联。
