Doede T, Wollina U, Hindermann W, Schier F, Bondartschuk M
Abteilung Kinderchirurgie, Klinikum der FSU Jena, Bachstrasse 18, 07740 Jena, Germany.
Pediatr Surg Int. 2003 Jul;19(5):406-8. doi: 10.1007/s00383-003-1020-7. Epub 2003 Jun 21.
Scleroderma can be differentiated into progressive systemic and circumscript forms. The extensive form with lethal outcome is known from case reports of children and adolescents. The present case report concerns a boy who died at 16 years of age. In the 5th year of life, he experienced weight loss and developed multiple, firm, partially atrophic plaques in the skin of the extremities. These plaques gradually became confluent and extended over the whole torso and head. Plaque ulceration resulted in massive mutilations to the body. Later the patient's cachexia worsened and he developed keratose, moderately differentiated squamous cell carcinoma of the right leg. The prognosis of pansclerotic morphea for children is worse than for adults. No successful therapy is known.
硬皮病可分为进行性系统性和局限性两种类型。儿童和青少年的病例报告中记载了具有致命结局的广泛性硬皮病。本病例报告讲述的是一名16岁死亡的男孩。在他5岁时，体重减轻，四肢皮肤出现多个坚硬、部分萎缩的斑块。这些斑块逐渐融合并蔓延至整个躯干和头部。斑块溃疡导致身体大面积毁损。后来患者恶病质加重，并患上了角化性、中度分化的右腿鳞状细胞癌。儿童泛发性硬斑病的预后比成人更差。目前尚无成功的治疗方法。
