Poggi P, Marchetti C, Calligaro A, Casasco A, Scelsi R
Institute of Histology and General Embryology, University of Pavia, Italy.
Lymphology. 1992 Dec;25(4):172-81.
Myotonic dystrophy is an hereditary disorder of several organ systems. Skeletal muscle is a principal target organ, but abnormalities also occur in the peripheral microcirculation. Because morphological and functional changes in the dermal blood microcirculation may affect interstitial fluid drainage of the skin, we examined dermal lymphatic morphology in adult patients with myotonic dystrophy. Skin biopsies were taken from the big toe from patients with myotonic dystrophy (age 18-50 years) and subjected to light and electron microscopy; five healthy subjects served as controls. The salient findings in myotonic dystrophy were ultrastructural changes of the lymphatic endothelial cells and the fibrillar elements that surround the lymphatic wall. These abnormal lymphatic findings are interpreted in light of changes in the blood microvasculature and loose connective tissue in this disorder.
强直性肌营养不良是一种累及多个器官系统的遗传性疾病。骨骼肌是主要的靶器官，但外周微循环也会出现异常。由于真皮血液微循环的形态和功能变化可能会影响皮肤的间质液引流，我们对成年强直性肌营养不良患者的真皮淋巴管形态进行了检查。从18至50岁的强直性肌营养不良患者的大脚趾处取皮肤活检组织，并进行光镜和电镜检查；选取五名健康受试者作为对照。强直性肌营养不良的显著发现是淋巴管内皮细胞以及围绕淋巴管壁的纤维成分的超微结构变化。根据该疾病中血液微血管和疏松结缔组织的变化对这些异常的淋巴管发现进行了解释。
