A Case of Localized Bullous Pemphigoid Associated with an Arteriovenous Fistula.

Sir,

Bullous pemphigoid (BP) is a chronic subepidermal bullous disease with an autoimmune etiology. It is caused by immunoglobulin G antibodies against hemidesmosomes and the lamina lucida of the basement membrane. Clinically, it presents as tense bullae on an erythematous base. BP usually presents in a generalized distribution, but the skin lesions may be localized in some cases.

BP was once thought to be an idiopathic disorder,[1] but growing data suggest many possible triggering factors, such as ultraviolet rays, medication, and skin injury.[2] Localized BP has been reported in the clinical settings of radiotherapy, surgical wounds, trauma, or burns.[3] In addition, patients with end-stage renal disorder (ESRD) undergoing hemodialysis have been described with some bullous dermatoses, including porphyria cutanea tarda and pseudoporphyria,[3] but there are only a few case reports of BP in patients with ESRD on hemodialysis.[4] Moreover, the localized form of BP is extremely rare; to our knowledge, only three cases have been reported in the English literature.[3]

A 77-year-old man with ESRD on hemodialysis was referred to the department of dermatology for recurrent bullae and erosion on his right hand for 6 weeks. On clinical examination, he had an arteriovenous fistula on the right forearm, and the erythematous tense bulla was limited to his hand, distal to the fistula. His right hand showed slight edema and distinct hyperpigmentation [Figure 1]. He had a 15-year history of type 2 diabetes mellitus, and his ESRD was due to diabetic nephropathy. He was treated with hemodialysis for last 7 years (thrice weekly sessions).

Figure 1

(a) Brown hyperpigmentation and mild edema of the dorsum of the right hand. The skin lesion was located in distal part of the arteriovenous fistula. (b) Localized erythematous vesicle (black arrow)

Laboratory findings revealed azotemia (creatinine, 11.21 mg/dL and eGFR, 3.341 ml/min) and eosinophilia (9.54 × 108/L). Hemoglobin A1c level was 6.5%. Histopathologic examination revealed subepidermal blister formation with an eosinophilic and lymphocytic infiltration in the upper dermis. Basal pigmentation and dermal hemosiderin deposition were also observed [Figure 2]. Immunoglobulin G and complement C3 deposition along the basement membrane zone was detected on DIF study [Figure 3]. IIF was positive at 1:320 dilution. From the histological findings and immunofluorescent study results, the patient was diagnosed with localized bullous pemphigoid.

Figure 2

(a) Subepidermal blister (H and E, ×12.5) (b) With eosinophilic infiltration (H and E, ×100). (c) Papillary dermis showed increased vascularity (H and E, ×40). (d) There were thickened capillaries with perivascular hemosiderin deposit (H and E, ×400)

Figure 3

Positive direct immunofluorescence staining for IgG (a) and C3 (b) along the basement membrane zone (Direct immunofluorescence staining, ×100)

Various clinical settings causing damage to the skin have been reported to cause localized BP, including trauma, surgical wound, irradiated site and sun-exposed area.[5] It has been postulated that the injury may reveal the BP antigen in immunologically susceptible individuals, resulting in antibody production and subsequent localized development of the lesions.

There are only seven case reports of BP associated with an arteriovenous fistula in hemodialysis patients.[4] Among them, two patients developed BP a short time after fistula placement for hemodialysis (3 days and 2 weeks), whereas BP occurred 1–11 years after the initiation of hemodialysis in five patients. Three patients had localized BP, and four patients developed the generalized type. Patients with localized BP were followed for one to two years. Herein, we report a rare case of localized bullous pemphigoid that developed in association with a hemodialysis fistula in a patient with end stage renal disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.