Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland.
Int Wound J. 2017 Feb;14(1):288-292. doi: 10.1111/iwj.12670. Epub 2016 Oct 17.
Haemodialysis is the most frequent form of renal replacement therapy (RRT) in patients with end-stage renal disorder (ESRD). Patients with ESRD frequently develop skin problems, mainly xerosis, pruritus and hyperpigmentation, as well as bullous diseases, mainly porphyria or pseudoporphyria and, in some cases, bullous pemphigoid (BP). BP is the most common autoimmune sub-epidermal blistering disease, and it predominantly affects elderly people. Clinically, BP is characterised by generalised pruritic, bullous eruptions and urticaria-like lesions. Usually, BP is an idiopathic disorder; however, in some cases, underlying internal disorders are present, like diabetes or neurological disorders. Herein, we present a 33-year-old man with ESRD, maintained on haemodialysis, who developed BP. There are only six cases with BP provoked by the placement of a fistula for haemodialysis. BP in the current patient was confirmed by direct immunofluorescence (DIF) and indirect immunofluorescence using BIOCHIP. The patient responded promptly to tertracycline and 0·05% clobetasol propionate lesionally. However, the relationship between BP and the fistula for haemodialisys still remains unknown. It is highly likely that the skin injury associated with fistula placement was responsible for the alteration of the basement membrane zone (BMZ) and the stimulation of the immune system, leading to BP development. To explain the real role of fistula placement as a provocative factor in BP, other such cases are required for assessment.
血液透析是终末期肾病(ESRD)患者最常用的肾脏替代治疗(RRT)形式。ESRD 患者常出现皮肤问题,主要为干燥、瘙痒和色素沉着,以及大疱性疾病,主要为卟啉病或假性卟啉病,在某些情况下还会出现大疱性类天疱疮(BP)。BP 是最常见的自身免疫性表皮下大疱性疾病,主要影响老年人。临床上,BP 表现为全身性瘙痒、水疱性皮疹和荨麻疹样病变。通常,BP 是一种特发性疾病;然而,在某些情况下,可能存在潜在的内部疾病,如糖尿病或神经疾病。在此,我们介绍了一名 33 岁的 ESRD 男性患者,接受血液透析治疗,并发 BP。仅有 6 例因放置血液透析瘘管而引发 BP 的病例报告。该患者的 BP 通过直接免疫荧光(DIF)和 BIOCHIP 间接免疫荧光检测得到确诊。患者对四环素和 0.05%丙酸氯倍他索局部治疗反应迅速。然而,BP 与血液透析瘘管之间的关系尚不清楚。很可能与瘘管放置相关的皮肤损伤导致了基底膜带(BMZ)的改变和免疫系统的刺激,从而引发 BP 的发生。为了解释瘘管放置作为 BP 诱发因素的真正作用,还需要评估其他类似病例。