From the University of Michigan Medical School, Ann Arbor, MI.
Department of Surgery, Michigan Medicine, University of Michigan, Ann Arbor, MI.
Ann Plast Surg. 2023 Jun 1;90(6):631-635. doi: 10.1097/SAP.0000000000003529. Epub 2023 Mar 8.
Stiff skin syndrome (SSS) is a rare cutaneous disorder characterized by cutaneous fibrosis resulting in the early onset of thickened and indurated skin, joint mobility restrictions, and contractures. We describe a father and son with familial SSS who presented with bilateral exertional pain and a confirmed diagnosis of chronic exertional compartment syndrome on 4-compartment pressure testing. Patients experienced restored functionality with bilateral 4-compartment fasciotomy. Chronic exertional compartment syndrome should be considered in the differential diagnosis of patients with SSS and chronic pain of the lower limbs.
硬皮病综合征(SSS)是一种罕见的皮肤疾病,其特征为皮肤纤维化,导致早期出现增厚和硬化的皮肤、关节活动受限和挛缩。我们描述了一对父子患有家族性 SSS 的病例,他们表现为双侧运动性疼痛,并在 4 compartment 压力测试中确诊为慢性运动性间隔综合征。患者接受双侧 4 compartment 筋膜切开术治疗后功能恢复。慢性运动性间隔综合征应在 SSS 患者和下肢慢性疼痛的鉴别诊断中考虑。