Turner Ryan B, Haynes Harley A, Granter Scott R, Miller Danielle M
Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
J Am Acad Dermatol. 2009 Jun;60(6):1052-7. doi: 10.1016/j.jaad.2008.09.059.
Cutis laxa (CL) is an inherited or acquired connective tissue disorder characterized clinically by loosely hanging skin folds. There is often preceding cutaneous inflammatory eruption (ie, urticaria, eczema, erythema multiforme), and there is frequently internal organ involvement of the gastrointestinal, urogenital, pulmonary, and cardiovascular systems. Histologically, there are degenerative changes in the dermal elastic fibers. Of the few reports on this rare disorder, authors have speculated about an immune-mediated destruction of elastic fibers, and monoclonal gammopathies, such as multiple myeloma or heavy chain deposition disease, have a recognized association with CL. We report an unusual case of rapidly progressing acquired CL associated with leukocytoclastic vasculitis, IgA myeloma, and an immune complex-mediated glomerulonephritis. Light microscopy of the lax skin revealed complete absence of elastic fibers in areas of vasculitis.
皮肤松弛症（CL）是一种遗传性或获得性结缔组织疾病，临床特征为皮肤褶皱松弛下垂。通常有前驱皮肤炎症性皮疹（如荨麻疹、湿疹、多形红斑），且常累及胃肠道、泌尿生殖系统、肺部和心血管系统等内脏器官。组织学上，真皮弹性纤维有退行性改变。在关于这种罕见疾病的少数报道中，作者推测弹性纤维是由免疫介导破坏的，单克隆丙种球蛋白病，如多发性骨髓瘤或重链沉积病，与CL有明确关联。我们报告了一例罕见病例，为快速进展的获得性CL，伴有白细胞破碎性血管炎、IgA骨髓瘤和免疫复合物介导的肾小球肾炎。对松弛皮肤进行光学显微镜检查发现，血管炎区域完全没有弹性纤维。
