Kolde G, Bachus R, Ludolph A C
Department of Dermatology, Virchow-Clinics, Humboldt-University of Berlin, Germany.
Lancet. 1996 May 4;347(9010):1226-7. doi: 10.1016/s0140-6736(96)90737-0.
Patients with sporadic amyotrophic lateral sclerosis (ALS) show disorganised collagen and elastin of the dermis. We looked for inflammatory alterations to cutaneous blood vessels.
Seven patients with sporadic ALS were investigated; five were confined to bed, but none had bedsores. Light and electron microscopy of skin showed an oedematous dermis with collagen fibrils of irregular diameter. Small blood vessels were characterised by duplicated basement membranes and deposition of beta-amyloid protein, the main component of the neuronal and non-neuronal amyloid deposits in Alzheimer's disease. These skin changes were seen in all degrees of disability, but none was found in age-matched and sex-matched controls.
The skin in ALS is characterised by a distinctive pattern of alterations of connective tissue and blood vessels. Examination of skin in an additional and easily accessible investigation which may help elucidate the pathogenesis of ALS.
散发性肌萎缩侧索硬化症（ALS）患者表现出真皮层胶原蛋白和弹性蛋白排列紊乱。我们研究了皮肤血管的炎症改变。
对7例散发性ALS患者进行了研究；其中5例卧床，但均未出现褥疮。皮肤的光镜和电镜检查显示真皮层水肿，胶原纤维直径不规则。小血管的特征是基底膜重复以及β-淀粉样蛋白沉积，β-淀粉样蛋白是阿尔茨海默病中神经元和非神经元淀粉样沉积物的主要成分。所有残疾程度的患者均出现这些皮肤变化，但在年龄和性别匹配的对照组中未发现。
ALS患者的皮肤特征为结缔组织和血管的独特改变模式。皮肤检查是一种额外且易于进行的检查，可能有助于阐明ALS的发病机制。
