Bauer E A, Tabas M
Department of Medicine, Washington University School of Medicine, St Louis, MO 63110.
Arch Dermatol. 1988 May;124(5):734-6.
The pathophysiology of tissue fragility in recessive dystrophic epidermolysis bullosa may be due in part to excessive destruction of interstitial collagens by a structurally altered, but catalytically active, form of human skin collagenase. Therapeutic attempts directed toward reducing the expression of this enzyme have resulted in clinical improvement in some patients with the disease.
隐性营养不良型大疱性表皮松解症中组织脆性的病理生理学部分可能归因于一种结构改变但具有催化活性的人皮肤胶原酶对间质胶原的过度破坏。针对减少这种酶表达的治疗尝试已使一些该疾病患者的临床症状得到改善。