Pansclerotic morphea in childhood: a case report
Pansclerotic morphea in childhood: a case report
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DOI：
10.1007/s00383-003-1020-7
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发表时间：
2003-07-01
影响因子：
1.8
通讯作者：
Bondartschuk, M
中科院分区：
文献类型：
--
作者：
Doede, T;Wollina, U;Bondartschuk, M
硬皮病可分为进行性系统性硬皮病和边缘性硬皮病。从儿童和青少年的病例报告中可以了解到具有致命后果的广泛形式。本病例报告涉及一名16岁时死亡的男孩。在5岁时，他体重下降，四肢皮肤出现了多个坚硬的部分萎缩性斑块。这些斑块逐渐融合并扩展到整个躯干和头部。菌斑溃烂导致身体大面积残缺。后来患者恶病质恶化，发展为右腿角化、中分化鳞状细胞癌。儿童全硬化性睡眠的预后比成人差。目前还没有成功的治疗方法。
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.