Alicea-Negrón Abdiel J, González-Barreto Reina M, González-Chávez Jose R
Medical School, University of Puerto Rico School of Medicine, San Juan, PRI.
Dermatology, Ponce Health Sciences University, Ponce, PRI.
Cureus. 2024 Aug 19;16(8):e67227. doi: 10.7759/cureus.67227. eCollection 2024 Aug.
Hailey-Hailey disease is an autosomal dominant disorder caused by a mutation in the ATP2C1 gene and characterized by recurrent blisters, erosions, and crust in intertriginous areas. Currently, there are no curative treatments for Hailey-Hailey disease, and therapeutic strategies are focused on controlling skin microbial colonization, infection, and inflammation. Recent efforts have aimed to find therapies that target the biochemical pathway involved in the pathogenesis of this disease. Several case reports indicate the use of different biological agents to achieve long-term remission in patients with recalcitrant Hailey-Hailey disease. Tumor necrosis factor-alpha inhibitors have been used to treat and maintain remission in recalcitrant Hailey-Hailey disease patients, but additional reporting and studies are required. In this case series, we report three cases of recalcitrant Hailey-Hailey disease whose lesions were successfully controlled with adalimumab.
黑利-黑利病是一种常染色体显性疾病,由ATP2C1基因突变引起,其特征为在皮肤褶皱部位反复出现水疱、糜烂及结痂。目前,尚无治愈黑利-黑利病的疗法,治疗策略主要集中于控制皮肤微生物定植、感染及炎症。近期研究致力于寻找针对该疾病发病机制中生化途径的疗法。几例病例报告显示,使用不同生物制剂可使难治性黑利-黑利病患者实现长期缓解。肿瘤坏死因子-α抑制剂已用于治疗难治性黑利-黑利病患者并维持缓解状态,但仍需更多报告及研究。在此病例系列中,我们报告了3例难治性黑利-黑利病患者,其皮损通过阿达木单抗成功得到控制。