Prevalence and presumptive triggers of localized bullous pemphigoid

Abstract

Pemphigoid diseases are a group of autoimmune bullous dermatoses (AIBD) caused by autoantibodies binding to distinct structural proteins of the dermal-epidermal junction. 1 Within this group, bullous pemphigoid (BP) is the most frequent and characterized by autoantibodies targeting the hemidesmosomal proteins BP180 (type XVII collagen, COL17) and/or BP230. 1 The vast majority of BP patients show a generalized disease. Less frequently, skin lesions may be restricted to one or a few sites, for which the term localized BP (LBP) was coined. Presumptive triggers that have been described to precede the onset of LBP include radiotherapy, thermal and chemical irritation, ultraviolet (UV) and photodynamic therapy, and local injury, such as pressure and surgical treatments. [2][3][4][5][6][7][8] Despite these insights, scant data on the prevalence of LBP has been reported. To address this knowledge gap, we retrospectively evaluated the prevalence of LBP.

Blisters and Milia around the Peritoneal Dialysis Catheter: A Case of Localized Bullous Pemphigoid

“…The classical clinical findings are widespread, tense, fluid-filled blisters on an erythematous or non-inflammatory base. The disease may start as a localized eruption in 15% to 30% of patients [ 2 ] although strictly localized forms are much less frequent, accounting for 2.5% of cases [ 1 ]. The majority of localized cases have a predilection for the lower extremities [ 2 ], but lesions in sites of prior radiation, lymphedema, surgical wounds, colostomy or urostomy stoma have rarely been described [ 3 ].…”

“…Bullous pemphigoid (BP) is mostly thought of as a generalized blistering eruption affecting the trunk and the proximal extremities. Although this is a correct picture of the disease, a number of patients with BP may have localizedcutaneous disease either persisting over a variable period of time or initially localized and followed by dissemination later on in the clinical course [ 1 ]. We report on the appearance of multiple tense blisters surrounding the exit site of a Tenckhoff catheter in a 79-year-old woman with end-stage renal disease in peritoneal dialysis.…”

Unmet Medical Needs in Chronic, Non-communicable Inflammatory Skin Diseases

“…The molecules targeted by BP autoantibodies are the two hemidesmosomal proteins type XVII collagen (COL17, also called BP180) and BP230, and the former molecule has been recognized to be the major autoantigen. Triggering factors for BP include ultraviolet rays and other radiation, burns, trauma, and regulatory T-cell dysfunction ( 209 – 211 ). Dipeptidyl peptidase-4 (DPP-4) inhibitors have recently gained attention as a cause of BP ( 212 – 214 ).…”

Unilateral and Localized Bullous Eruption in a 71-year-old Woman

“…This variant is uncommon, with a recent cohort study suggesting a prevalence of 2.5% among BP patients. 1 LBP often arises in areas of previous trauma or radiation and has been more frequently reported in the pretibial, vulvar, peristomal, and umbilical regions. 2 The disease course is more benign than generalized BP due to its responsiveness to topical steroids, though LBP may also progress to generalized disease.…”

“…3,4 Triggering factors implicated in the development of LBP include radiation, burns, surgical procedures, phototherapy, venous stasis, and bacterial infections. 1 Several medications (e.g., angiotensin-convertingenzyme inhibitors, loop diuretics, gabapentin, iodine, dithranol, and tar) have also been reported to cause this variant. 5 In our case, immunotherapy was the likely causative factor leading to the development of LBP.…”