家族性良性天疱疮的治疗进展

Advances in the treatment of benign familial pemphigus

家族性良性天疱疮是一种少见的常染色体显性遗传性疾病,由ATP2C1基因突变所致。临床上病情反复,治疗困难,目前主要有药物治疗、外科治疗和激光治疗。药物治疗包括糖皮质激素、抗生素、维A酸类、维生素D3类似物、他克莫司和肉毒毒素等。为防止复发和延长缓解时间,可选用外科治疗和激光治疗。抗白介素-6抗体等生物学疗效有望治疗本病。

Benign familial pemphigns （HHD） is a rare autosomal dominant disease caused by mutations in the ATP2Clgene encoding a novel Ca^2＋ pump. It is a refractory skin disease with recurrent characteristic lesions. At present, its therapeutic options include medications, surgical intervention and laser therapy. Corticostemids, antibiotics, retinoids, vitamin D3 analogues, tacrolimus and botulinal toxin are commonly used in its treatment. In order to prolong remission and prevent relapse, surgical intervention and laser therapy are recommended. Biological therapy, such as anti IL-6 antibody, has a good prospect in the treatment of HHD.