Bullous Pemphigoid: A Review of its Diagnosis, Associations and Treatment

Abstract

Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries, and typically affects the elderly. BP is immunologically characterized by tissue-bound and circulating autoantibodies directed against either the BP antigen 180 (BP180, or BPAG2) or the BP antigen 230 (BP230, or BPAG1e), or even both, which are components of hemidesmosomes involved in the dermal-epidermal cohesion. Risk factors for BP include old age, neurologic diseases (dementia, Parkinson's disease, cerebrovascular disease), and some particular drugs, including loop diuretics, spironolactone and neuroleptics. The spectrum of clinical presentations is extremely broad. Clinically, BP is an intensely pruritic erythematous eruption with widespread blister formation. In the early stages, or in atypical, non-bullous variants of the disease, only excoriated, eczematous or urticarial lesions (either localized or generalized) are present. The diagnosis of BP relies on immunopathologic findings, especially based on both direct and indirect immunofluorescence microscopy observations, as well as on anti-BP180/BP230 enzyme-linked immunosorbent assays (ELISAs). BP is usually a chronic disease, with spontaneous exacerbations and remissions, which may be accompanied by significant morbidity. In the past decade, potent topical corticosteroids have emerged as an effective and safe first-line treatment for BP, but their long-term feasibility is still controversial. Newer therapeutic agents targeting molecules involved in the inflammatory cascade associated with BP represent future alternatives to classical immunosuppressant drugs for maintenance therapy.

Localized bullous pemphigoid: Four clinical cases and a literature review

“…They are henceforth also more likely to develop BP‐type illnesses. Old age or specific medications (neuroleptics, spironolactones, loop diuretics, etc) are also mentioned as risk factors for developing LBP or BP . Differential diagnosis is essential for contact dermatitis, irritative dermatitis, other bullous illnesses, and viral infections, such as the varicella‐zoster virus or herpes.…”

The spectrum of histopathologic findings in pemphigoid: Avoiding diagnostic pitfalls

“…It is possible that issues related to the re‐epithelialization of a subepidermal blister apply to a range of diseases other than BP. In fact, epidermolysis bullosa acquisita (EBA) can be quite difficult to distinguish because the clinical and histological features overlap considerably and both show linear deposition of IgG and C3 on DIF . The difference is the immunologic target: hemidesmosomes located above the lamina densa are targeted in BP whereas collagen VII below the lamina densa in the basement membrane is the target in EBA.…”

Targeting interleukin 4 receptor α: A new approach to the treatment of cutaneous autoimmune bullous diseases?

“…Therefore, newer therapeutic agents which could be more selective would represent a new treatment horizon for BP (Bernard & Antonicelli, 2017). Currently, the treatment relies on corticosteroids and immunosuppressant drugs.…”