Epidermolysis bullosa dystrophica dominans – ein Defekt der anchoring fibrils

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Abstract
Ultrastructural studies on a familiar case of Epidermolysis bullosa dystrophica dominans have demonstrated that normal anchoring fibrils are missing in the junctional area beneath the basal lamina; some rudimentary fibrils without typical banding patterns may be found occasionally. All other junctional structures, i.e. half desmosomes, plasmamembrane, anchoring filaments, and basal lamina, are of normal ultra-structure. The bulla is formed subepidermally, the basal lamina remaining at the top of the bulla.

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