Department of Dermatology, University Medical Center Freiburg, Freiburg, Germany.
Dermatology. 2010;221(2):122-6. doi: 10.1159/000314155. Epub 2010 Jun 26.
Pemphigus vulgaris (PV) is an auto-immune blistering skin disease characterized by flaccid blisters and painful erosions of mucous membranes and skin. Suprabasal blister formation results from a loss of epidermal cohesion induced by auto-antibodies against the desmosomal protein desmoglein 3. Treatment of PV currently consists of systemic glucocorticosteroids and adjuvant immunosuppressive drugs such as azathioprine, mycophenolate mofetil, cyclophosphamide or dapsone. Due to the low incidence of PV, there are insufficient data to conclude which treatment is the most effective and safest. Thus far, systemic tacrolimus (FK506, Prograf) has not yet been reported as adjuvant medication for PV. Here, we describe the successful use of systemic tacrolimus in 2 patients with recalcitrant PV of the oral mucosa. Tacrolimus was well tolerated, and clinical improvement allowed tapering of corticosteroids. Thus, oral tacrolimus may be a therapeutic alternative for patients with recalcitrant PV.
寻常型天疱疮（PV）是一种自身免疫性水疱性皮肤病，其特征为皮肤和黏膜出现松弛性水疱和疼痛性糜烂。桥粒芯糖蛋白 3 的自身抗体导致表皮细胞间黏附丧失，从而引发超基底水疱形成。目前 PV 的治疗方法包括全身糖皮质激素和辅助免疫抑制剂，如硫唑嘌呤、霉酚酸酯、环磷酰胺或氨苯砜。由于 PV 的发病率较低，因此尚无足够的数据来确定哪种治疗方法最有效和最安全。到目前为止，尚无报道称全身他克莫司（FK506，普乐可复）可作为 PV 的辅助药物。在这里，我们描述了 2 例口腔黏膜难治性 PV 患者使用全身他克莫司治疗的成功经验。他克莫司耐受性良好，临床改善后可逐渐减少皮质类固醇的用量。因此，口服他克莫司可能是治疗难治性 PV 的一种治疗选择。
