Cho S Y, Maguire R F
Cutis. 1980 Aug;26(2):209-11.
Acquired cutis laxa is a rare disorder characterized by diffuse laxity of the skin and loss of connective tissue support with involvement of the lungs, gastrointestinal tract, pelvic organs, and aorta. The case report presented herein describes a forty-six year old woman with multiple myeloma and cutis laxa. Her history included several severe allergic reactions and the gradual development of lax skin, loss of connective tissue support throughout the body, and emphysema. At autopsy, multiple myeloma, diffuse laxity of the skin, and panacinar emphysema were found. The amount of elastic fiber in the skin, lungs, and aorta was decreased and showed abnormal fragmentation. Results of direct immunofluorescence study demonstrated IgG bound to dermal elastic fibers. Speculation regarding an immunologic etiology of the elastic tissue abnormality is presented herein.
获得性皮肤松弛症是一种罕见的疾病,其特征为皮肤弥漫性松弛以及结缔组织支撑丧失,并累及肺部、胃肠道、盆腔器官和主动脉。本文呈现的病例报告描述了一名患有多发性骨髓瘤和皮肤松弛症的46岁女性。她的病史包括几次严重的过敏反应以及皮肤逐渐松弛、全身结缔组织支撑丧失和肺气肿。尸检发现多发性骨髓瘤、皮肤弥漫性松弛和全腺泡型肺气肿。皮肤、肺和主动脉中的弹性纤维数量减少并显示出异常断裂。直接免疫荧光研究结果表明IgG与真皮弹性纤维结合。本文提出了关于弹性组织异常的免疫病因学推测。