Department of Dermatology, Medical Center- University of Freiburg, Faculty of Medicine, University of Freiburg, Hauptstr. 7, 79104, Freiburg, Germany.
Royal Melbourne Hospital, Parkville and Box Hill Hospital - Monash University Eastern Health Clinical School, Box Hill, VIC, Australia.
Orphanet J Rare Dis. 2018 Sep 18;13(1):163. doi: 10.1186/s13023-018-0899-y.
IgA pemphigus is an exceedingly rare autoimmune blistering disorder, caused by IgA autoantibodies against desmosomal proteins. No treatment option has been found to be universally effective. The disease is often recalcitrant to oral steroids and immunosuppressants. Here, we describe the use of systemic retinoids for the treatment of recalcitrant IgA pemphigus in 3 cases. Although the use of acitretin has been reported before, we present for the first time the positive effects of alitretinoin in treatment of 2 patients with IgA pemphigus. Besides hyperlipoproteinaemia requiring use of hypolipidemic agents in one case, alitretinoin was well-tolerated and has generally a more favorable side effect spectrum than immunosuppressants.
IgA 天疱疮是一种极为罕见的自身免疫性水疱病，由针对桥粒蛋白的 IgA 自身抗体引起。目前还没有发现一种普遍有效的治疗方法。这种疾病通常对口服类固醇和免疫抑制剂有抗药性。在这里，我们描述了用系统类视黄醇治疗 3 例难治性 IgA 天疱疮的情况。虽然以前曾报道过阿维 A 的使用，但我们首次报告了阿利维 A 在治疗 2 例 IgA 天疱疮中的积极作用。除了 1 例需要使用降脂药来治疗高脂血症外，阿利维 A 耐受性良好，其副作用谱通常比免疫抑制剂更有利。
