Barata Rui, Moreira Fonseca Nuno, Assis Pereira Tiago, Góis Mário, Sousa Helena, Carvalho Dulce, Ribeiro Francisco, Fernandes Cândida, Pena Ana, Nolasco Fernando
Service de néphrologie, Centro Hospitalar Universitário de Lisboa Central, R. Beneficência 8, 1050-099 Lisboa, Portugal.
Service de néphrologie, Centro Hospitalar Universitário de Lisboa Central, R. Beneficência 8, 1050-099 Lisboa, Portugal.
Nephrol Ther. 2021 Dec;17(7):547-551. doi: 10.1016/j.nephro.2021.07.001. Epub 2021 Oct 8.
Bullous pemphigoid is an autoimmune bullous cutaneous disease. We report the case of a 60 year-old male patient whose kidney allograft failed and was on hemodialysis for the previous 16 months. After tapering immunosuppressive medication, he presented simultaneous bullous eruption and kidney allograft intolerance syndrome. Investigation showed a positive BP180 anti-basement membrane zone antibody and skin biopsy was consistent with bullous pemphigoid. The patient was treated with corticotherapy and bullous pemphigoid resolved. The development of new onset diabetes and concerns over long term immunosuppression, halted the decision to continue corticotherapy and the patient underwent graft nephrectomy, with resolution of the kidney allograft intolerance syndrome without recurrence of the bullous disease. The occurrence of bullous pemphigoid in patients with failed renal allograft is rare, with only eleven cases reported in literature. This case illustrates how graft nephrectomy can provide a definitive cure to bullous pemphigoid in this setting.
大疱性类天疱疮是一种自身免疫性大疱性皮肤病。我们报告了一例60岁男性患者，其同种异体肾移植失败，在过去16个月里一直进行血液透析。在逐渐减少免疫抑制药物用量后，他同时出现了大疱性皮疹和同种异体肾移植不耐受综合征。检查显示BP180抗基底膜带抗体呈阳性，皮肤活检结果与大疱性类天疱疮相符。该患者接受了皮质激素治疗，大疱性类天疱疮得以缓解。新发糖尿病的出现以及对长期免疫抑制的担忧，使得继续进行皮质激素治疗的决定被搁置，患者接受了移植肾切除术，同种异体肾移植不耐受综合征得以解决，大疱性疾病未复发。肾移植失败患者发生大疱性类天疱疮的情况罕见，文献中仅报道了11例。该病例说明了在这种情况下，移植肾切除术如何能为大疱性类天疱疮提供确切的治愈方法。
