Brinkmeier Thomas, Angelkort Bernd, Frosch Peter J, Herbst Rudolf A
Hautklinik, Klinikum Dortmund gGmbH und Universität Witten/Herdecke.
J Dtsch Dermatol Ges. 2003 Mar;1(3):212-4. doi: 10.1046/j.1610-0387.2003.02037.x.
Linear IgA disease is a rare autoimmune bullous skin disorder that may be idiopathic or drug-induced. The mechanisms of loss of self-tolerance to target antigens of the dermo-epidermal junction are unclear and may be variable. Regardless of the etiology, the clinical presentation is quite heterogeneous. Frozen sections of a blister roof are helpful in early diagnosis to rule out full-thickness epidermal necrosis or subcorneal separation as seen in toxic epidermal necrosis or staphylococcal scaled skin syndrome, respectively. Drug-induced cases of linear IgA disease typically resolve quickly once the causative agent is withdrawn. Vancomycin is the best-documented insiting drug in the literature. The significance of further associations such as malignancy or different autoimmune diseases is uncertain. Review of medication exposures after diagnosis is crucial in identifying possible offending agents. We present a patient with vancomycin-induced linear IgA disease in whom the drug was immediately discontinued because of the characteristic clinical presentation and rapid histopathological examination. The development of new blisters stopped within 24 hours and the skin healed within 2 weeks.
线状IgA疾病是一种罕见的自身免疫性大疱性皮肤病,可能是特发性的或药物诱导的。对真皮-表皮交界处靶抗原的自身耐受丧失机制尚不清楚,且可能各不相同。无论病因如何,临床表现都非常异质性。水疱顶部的冰冻切片有助于早期诊断,以排除分别在中毒性表皮坏死或葡萄球菌性烫伤样皮肤综合征中所见的全层表皮坏死或角质层下分离。一旦停用致病药物,药物诱导的线状IgA疾病病例通常会迅速缓解。万古霉素是文献中记载最充分的致病药物。恶性肿瘤或不同自身免疫性疾病等进一步关联的意义尚不确定。诊断后回顾药物暴露情况对于确定可能的致病因素至关重要。我们报告一例万古霉素诱导的线状IgA疾病患者,因其特征性临床表现和快速组织病理学检查,药物立即停用。新水疱的形成在24小时内停止,皮肤在2周内愈合。