Valverde Ricardo, Rosales Belén, Ortiz-de Frutos Francisco Javier, Rodríguez-Peralto José Luis, Ortiz-Romero Pablo L
"Hospital Universitario Doce de Octubre", Universidad Complutense, Madrid, Spain.
Dermatol Clin. 2008 Oct;26(4):447-51, vi. doi: 10.1016/j.det.2008.05.001.
Alpha-1-antitrypsin deficiency is a congenital error of metabolism linked to pulmonary (emphysema) and liver (cirrhosis) disease. Since 1972, panniculitis has been associated with this deficiency, initially related to Weber-Christian syndrome and finally as a differentiated entity. Clinical manifestations typically consist of wide nodular lesions on the trunk and proximal extremities that evolve to ulceration and drainage. Histopathologically it presents as a mixed septal-lobular panniculitis pattern with some typical findings referred. Differential diagnosis from other types of panniculitis and neutrophilic dermatosis must be established. Different treatments, including tetracyclines, dapsone, and alpha-1-antitrypsin repositioning, have shown variable efficacy in controlling this disease.
α-1抗胰蛋白酶缺乏症是一种与肺部（肺气肿）和肝脏（肝硬化）疾病相关的先天性代谢紊乱。自1972年以来，脂膜炎一直与这种缺乏症相关，最初与韦氏-克里斯蒂安综合征有关，最终成为一种独立的疾病实体。临床表现通常为躯干和近端肢体出现广泛的结节性病变，并发展为溃疡和渗液。组织病理学表现为混合性间隔小叶性脂膜炎模式，并伴有一些典型表现。必须与其他类型的脂膜炎和嗜中性皮病进行鉴别诊断。不同的治疗方法，包括四环素、氨苯砜和α-1抗胰蛋白酶替代治疗，在控制这种疾病方面显示出不同的疗效。
