Liszewski Walter, Omland Silje Haukali, Gniadecki Robert
Department of Dermatology, Bispebjerg Hospital, University of Copenhagen, Copenhagen, Denmark.
Pediatr Dermatol. 2015 Mar-Apr;32(2):e60-1. doi: 10.1111/pde.12526. Epub 2015 Jan 30.
Epidermolysis bullosa acquisita is a rare autoimmune bullous disease of the mucosa and skin characterized by the presence of anti-collagen VII antibodies at the dermoepidermal junction. Most patients respond to immunosuppressive or antiinflammatory agents, although patients whose condition is refractory to these therapies will require more aggressive treatment. We present a 12-year-old girl with refractory epidermolysis bullosa acquisita who responded to extracorporeal photopheresis.
获得性大疱性表皮松解症是一种罕见的黏膜和皮肤自身免疫性大疱病,其特征是在真皮表皮交界处存在抗VII型胶原蛋白抗体。大多数患者对免疫抑制剂或抗炎药有反应,尽管对这些治疗无效的患者需要更积极的治疗。我们报告了一名12岁患有难治性获得性大疱性表皮松解症的女孩,她对体外光化学疗法有反应。