Development of bullous pemphigoid during the haemodialysis of a young man: case report and literature survey

Abstract

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.

Development of Bullous Pemphigoid after Tenckhoff Catheter Placement in a Peritoneal Dialysis Patient

“…We suppose that these lesions were already due to the pemphigoid and that the catheter placement together with the skin injury due to the daily plaster removal for the medication of the exit site could have represented a traumatic “second-hit”. In the literature 6 cases are described [10] of new BP onset after the placement of an artero-venous fistula. To the best of our knowledge, this is the first case describing a new BP onset after a peritoneal dialysis catheter placement.…”

Development of generalized bullous lesions after hemodialysis with polysulfone membrane dialyzer

“…Dear Editor, To date, a few cases of hemodialysis-associated bullous pemphigoid (BP) have been reported in relation to the hemodialysis fistula. 1 Herein, we present a rare case of generalized BP associated with polysulfone membrane dialyzer. A 46-year-old man developed tense bullae approximately 6-8 h after the first hemodialysis with a polysulfone membrane dialyzer (REXEED-13LC; Asahi Kasei, Gobo, Japan).…”

Laboratory Diagnosis of Porphyria

“…On the contrary, patients undergoing maintenance hemodialysis therapy [22,23] or those co-infected with immunodeficiency virus (HIV) and hepatitis C virus (HCV) [24] may develop a secondary elevation of plasma porphyrin along with cutaneous lesions, similar to those observed in genetic PCT patients, and as a consequence, a characteristic fluorescence peak appears at 618-620 nm. Therefore, this test might be useful for monitoring the activity of all the conditions referred to as pseudoporphyria, which do not result from an enzymatic absence of genetic origin.…”