Kennemer Caroline, Pavlidakey Peter, Sami Naveed
School of Medicine, The University of Alabama at Birmingham, Birmingham, Alabama.
Department of Dermatology, The University of Alabama at Birmingham, Birmingham, Alabama.
Dermatol Ther. 2017 Jul;30(4). doi: 10.1111/dth.12504. Epub 2017 Jun 2.
Scleredema is a deposition disorder which presents as diffuse, symmetric, non-pitting skin induration from dermal deposits of glycosaminoglycans ("mucins"). It classically affects the upper back and posterior neck, often causing skin tightness and decreased range of motion. In most patients the clinical course is chronic and treatment options are limited. We report a case of a patient diagnosed with scleredema associated with longstanding insulin dependent diabetes who was treated successfully with IVIg. Additionally, we have reviewed the literature reporting other patients with scleredema treated with IVIg.
硬化性水肿是一种沉积性疾病,表现为因糖胺聚糖(“黏蛋白”)在真皮沉积而导致的弥漫性、对称性、非凹陷性皮肤硬结。典型表现为累及上背部和后颈部,常导致皮肤紧绷和活动范围受限。大多数患者的临床病程呈慢性,治疗选择有限。我们报告一例诊断为与长期胰岛素依赖型糖尿病相关的硬化性水肿患者,经静脉注射免疫球蛋白治疗成功。此外,我们还回顾了报道其他接受静脉注射免疫球蛋白治疗的硬化性水肿患者的文献。