Watanabe S, Yamada K, Ono S, Ishibashi Y
Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
J Am Acad Dermatol. 1987 Dec;17(6):1006-12. doi: 10.1016/s0190-9622(87)70290-4.
Patients with amyotrophic lateral sclerosis are usually confined to bed within a few years from the onset of the disease; however, bedsores are not known to develop even at its terminal stages. In this study, skin from patients with amyotrophic lateral sclerosis, neuromuscular diseases other than amyotrophic lateral sclerosis, and nonneurologic disorders has been examined by light and electron microscopy for morphologic changes in the connective tissue. On physical examination of the skin in amyotrophic lateral sclerosis patients, the stretched fold showed a delayed relaxation phase. On light microscopy, collagen bundles in the dermis were disoriented, separated, and fragmented, especially in the papillary dermis. Ultrastructurally, extensive deposits of amorphous, fine granular material in the ground substance compartment of the dermis were observed. The preceding cutaneous changes became more apparent as the disease advanced. We speculate that the deposition of this amorphous material may play the role of pressure absorber, thus preventing the occlusion of blood vessels.
肌萎缩侧索硬化症患者通常在疾病发作后的几年内就会卧床不起；然而，即使在疾病晚期也未见褥疮形成。在本研究中，通过光镜和电镜检查了肌萎缩侧索硬化症患者、除肌萎缩侧索硬化症外的神经肌肉疾病患者以及非神经疾病患者的皮肤，以观察结缔组织的形态学变化。在对肌萎缩侧索硬化症患者的皮肤进行体格检查时，拉伸褶皱显示出延迟的松弛期。光镜下，真皮中的胶原束排列紊乱、分离且断裂，尤其是在乳头层真皮中。超微结构观察发现，真皮基质区有大量无定形、细颗粒状物质沉积。随着疾病进展，先前的皮肤变化变得更加明显。我们推测这种无定形物质的沉积可能起到了压力吸收器的作用，从而防止血管闭塞。
