α-1抗胰蛋白酶缺乏症与脂膜炎。疾病关系及替代疗法的观点。
Alpha-1-antitrypsin deficiency and panniculitis. Perspectives on disease relationship and replacement therapy.
作者信息
Pittelkow M R, Smith K C, Su W P
机构信息
Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905.
出版信息
Am J Med. 1988 Jun 24;84(6A):80-6. doi: 10.1016/0002-9343(88)90163-5.
A distinctive form of ulcerative panniculitis develops in a subset of persons with alpha-1-antitrypsin deficiency. This association may be more widely recognized if routine determinations of alpha-1-antitrypsin are performed on patients in whom neutrophilic, ulcerative panniculitis develops without specific, defined underlying causes. Therapies that appear to be of the greatest potential benefit include treatment with dapsone and alpha-1-proteinase inhibitor replacement. Astute recognition of alpha-1-antitrypsin-deficient panniculitis and assessment of response to these and other therapies will ultimately lead to a more complete understanding of the cause of this distinctive and uniquely associated disease.
一种独特的溃疡性脂膜炎在一部分α-1抗胰蛋白酶缺乏症患者中发生。如果对发生中性粒细胞性、溃疡性脂膜炎且无特定明确潜在病因的患者进行α-1抗胰蛋白酶的常规检测，这种关联可能会得到更广泛的认识。似乎最具潜在益处的治疗方法包括使用氨苯砜治疗和α-1蛋白酶抑制剂替代治疗。对α-1抗胰蛋白酶缺乏性脂膜炎的敏锐识别以及对这些和其他治疗反应的评估最终将有助于更全面地了解这种独特且具有独特关联疾病的病因。
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