Collagenase and connective tissue metabolism in epidermolysis bullosa.
Collagenase and connective tissue metabolism in epidermolysis bullosa.
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大疱性表皮松解症中的胶原酶和结缔组织代谢。
DOI：
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发表时间：
1972
影响因子：
6.5
通讯作者：
G. Lazarus
中科院分区：
文献类型：
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作者：
G. Lazarus
研究了19例不同类型的大疱性表皮病患者的胶原蛋白增加的证据。9例皮炎性大疱性皮肤病患者、4例单纯性表皮大疱性皮肤病患者和2例交界性大疱性皮肤病患者的临床正常但病理可破坏的皮肤培养物在胶原酶产生方面没有显著差异。在两个患皮炎性大疱性皮肤病的患者中，皮损培养物产生的胶原酶是邻近正常外观但病理上可破坏皮肤的三倍，DNA含量是其两倍。9例皮炎性大疱性皮肤病患者中有2例血清胶原酶抑制剂水平极轻微降低。没有患者尿中羟脯氨酸排泄增加。6例皮炎性大疱性皮肤病患者中有4例和2例交界性大疱性皮肤病患者中有1例有抗变性胶原抗体。似乎局部胶原酶水平升高是慢性损伤的继发性组织反应。
Ninteen patients with various types of epidermolysis bullosa were studied for evidence of increased collagen catabolism. Cultures of clinically normal but pathologically blisterable skin from nine patients with dermolytic bullous dermatoses, four patients with epidermolysis bullosa simplex and two patients with junctional bullous dermatoses did not differ significantly in collagenase production. In two patients with dermolytic bullous dermatoses cultures of skin lesions produced three times more collagenase and had twice the DNA content of adjacent normal appearing but pathologically blisterable skin. Two of the nine patients with dermolytic bullous dermatoses had minimally decreased levels of serum collagenase inhibitor. None of the patients had increased hydroxyproline excretion in the urine. Four of six patients with dermolytic bullous dermatoses and one of two patients with junctional bullous dermatoses had anti-denatured collagen antibodies. It appears that increased local levels of collagenase are a secondary tissue reaction to chronic injury.