Katarincic J A, Bishop A T, Wood M B
Division of Hand Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
J Pediatr Orthop. 2000 Mar-Apr;20(2):255-8.
Linear scleroderma is a subtype of localized scleroderma that most commonly affects children. The disease is rare, and the course is unpredictable. Most cases spontaneously resolve in 3-5 years. A small number, however, slowly progress to involve deeper subcutaneous tissues, muscles, and periosteum. Subsequent joint contractures and gross disturbances are frequent. Little on treatment is recorded in the literature. We report on four children who, in addition to bony procedures and release of joint and ligamentous contractures, had excision of the diseased skin and free soft-tissue transfer for coverage.
线状硬皮病是局限性硬皮病的一种亚型,最常见于儿童。这种疾病较为罕见,病程难以预测。大多数病例会在3至5年内自行缓解。然而,少数病例会缓慢进展,累及更深层的皮下组织、肌肉和骨膜。随后频繁出现关节挛缩和严重功能障碍。文献中关于治疗的记载很少。我们报告了4例儿童病例,这些患儿除了接受骨手术以及松解关节和韧带挛缩外,还进行了病变皮肤切除和游离软组织转移覆盖术。