Shekhonin B V, Semiachkina A N, Makkaev Kh M, Domogatskiĭ S P, Idel'son G L
Arkh Patol. 1988;50(12):41-8.
Immunofluorescence was employed to study distribution of collagen type I, III, IV and V, and fibronectin in skin biopsy specimens from patients with Ehlers-Danlos syndrome (EDS) and cutis laxa. Abnormal distribution of collagen type V in the skin biopsies was found out in EDS type VII. A high level of dermal fibronectin occurred in EDS type III. Tissue fibronectin was absent in the skin extracellular matrix in EDS type X. Defective distribution of collagen, predominantly of type III and V and fibronectin absence were registered in the skin connective tissue in cutis laxa.
采用免疫荧光法研究了埃勒斯-当洛综合征(EDS)和皮肤松弛症患者皮肤活检标本中I、III、IV和V型胶原蛋白以及纤连蛋白的分布情况。在VII型EDS患者的皮肤活检中发现V型胶原蛋白分布异常。III型EDS患者真皮纤连蛋白水平较高。X型EDS患者皮肤细胞外基质中不存在组织纤连蛋白。在皮肤松弛症患者的皮肤结缔组织中,胶原蛋白分布存在缺陷,主要是III型和V型胶原蛋白,且不存在纤连蛋白。