Nikko A, Dunnigan M, Black A, Cockerell C J
Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, USA.
Am J Dermatopathol. 1996 Oct;18(5):533-7. doi: 10.1097/00000372-199610000-00013.
Acquired cutis laxa (generalized acquired elastolysis) is characterized by skin laxity often with systemic involvement of the lungs, aorta, gastrointestinal tract, and pelvic organs. Although there have been many speculations regarding its pathogenesis, the etiology of this condition is still unclear. We describe a patient with generalized acquired cutis laxa associated with a plasma cell dyscrasia. Immunofluorescence studies were used to evaluate the pathogenesis of the elastolysis. Lesional skin was examined by direct and indirect immunofluorescence techniques for evidence of deposition of immunoglobulins on elastic fibers in the dermis. Direct immunofluorescence revealed deposition of IgG on elastic fibers in the dermis. Some patients with acquired cutis laxa have underlying lymphoreticular disorders such as plasma cell dyscrasia and may have immunoglobulins deposited on dermal elastic fibers. Immune-mediated mechanisms may play a major role in the pathogenesis of acquired cutis laxa in at least some cases.
获得性皮肤松弛症(全身性获得性弹性组织离解)的特征是皮肤松弛,常伴有肺部、主动脉、胃肠道和盆腔器官的全身受累。尽管对其发病机制有许多推测,但这种病症的病因仍不清楚。我们描述了一名患有与浆细胞异常增生相关的全身性获得性皮肤松弛症的患者。免疫荧光研究用于评估弹性组织离解的发病机制。通过直接和间接免疫荧光技术检查病变皮肤,以寻找免疫球蛋白在真皮弹性纤维上沉积的证据。直接免疫荧光显示真皮弹性纤维上有IgG沉积。一些获得性皮肤松弛症患者有潜在的淋巴网状系统疾病,如浆细胞异常增生,且免疫球蛋白可能沉积在真皮弹性纤维上。免疫介导机制可能在至少某些病例的获得性皮肤松弛症发病机制中起主要作用。