Ultrastructural studies in epidermolysis bullosa hereditaria

摘要: Ultrastructural examination was performed in 42 biopsy specimens from 22 patients with the Hallopeau-Siemens types or inverse type of epidermolysis bullosa dystrophica recessiva. The patient group consists 8 cases localized type, 9 generalized and 5 type. origins are involved, intact experimentally frictioned skin blister-predilected sites, as well clinically normal nonpredilection sites. It is confirmed that all blisters initiate below basal lamina. Anchoring fibrils moderately to markedly decreased most cases, but 3 cases. collagen more less changed majority while they other thought secondary degradation anchoring and/or plays an important role blistering mechanism recessive dystrophic bullosa, whereas a primary aplasia causative defect has been out ruled.