Pemphigoid Diseases Affecting the Skin

Abstract

Pemphigoid diseases are a heterogeneous group of subepidermal autoimmune blistering diseases (sAIBD) that are characterized by autoantibodies against different structural proteins of hemidesmosomes in the epidermal basement membrane zone (EBMZ). The group of pemphigoid diseases affecting the skin include various subtypes, such as bullous pemphigoid (BP), nonbullous pemphigoid (NBP), Brunsting-Perry cicatricial pemphigoid, lichen planus pemphigoides (LPP), pemphigoid gestationis (PG), and anti-p200 pemphigoid. Classification of sAIBD subtypes is mainly based on target antigens and/or clinical manifestations. Pathogenesis of pemphigoid diseases is mediated by predominantly IgG autoantibodies against different structural proteins in the EBMZ. Diagnosis is based on a combination of clinical features, an n-serrated linear pattern of immunodepositions along the EBMZ in direct immunofluorescence microscopy and immunoserology. BP is the most common subtype and most frequently affects elderly, the incidence of BP increased substantially in the past decades. The clinical manifestations of pemphigoid diseases are heterogeneous and represent a clinical spectrum. The typical presentation of BP is a severe pruritus with a predominantly cutaneous lesions consisting of tense blisters or vesicles, erythema and urticarial plaques. In NBP blistering is absent, while the pruritus is severe, and erythematous papules, plaques and excoriations may mimick other inflammatory dermatoses. Recommended therapies consists of whole-body application of superpotent topical corticosteroids, or systemic treatment with immunosuppressive or -modulating drugs.