Pemphigoid diseases are a heterogeneous group of subepidermal autoimmune bullous diseases (sAIBDs) that are characterized by autoantibodies against different structural proteins of hemidesmosomes in the epidermal basement membrane zone (EBMZ) (Table. 14.1). Cutaneous pemphigoid includes many subtypes, such as bullous pemphigoid (BP), nonbullous cutaneous pemphigoid (NBCP), Brunsting-Perry cicatricial pemphigoid, lichen planus pemphigoides (LPP), pemphigoid gestationis (PG), anti-p200 pemphigoid, and anti-plectin pemphigoid. Classification of sAIBD subtypes is mainly based on target antigens and/or clinical manifestations. Pathogenesis of cutaneous pemphigoid is mediated by predominantly IgG autoantibodies against different structural proteins in the EBMZ. Diagnosis is based on a combination of clinical criteria, a linear n-serrated deposition pattern along the EBMZ in direct immunofluorescence microscopy and serology. BP is the most common sAIBD and most frequently affects elderly. The incidence of BP increased substantially in the past decades. The clinical manifestations of pemphigoid diseases are heterogeneous. The typical presentation of BP is a severe pruritus with predominantly cutaneous lesions consisting of tense blisters or vesicles, erythema, and urticarial plaques. In NBCP, blistering is completely absent, while pruritus is severe, and papules, plaques, and excoriations may be present. Mucosal lesions develop in 10–20 % of patients. Nikolsky’s sign is negative in pemphigoid. Recommended therapy consists of whole-body application of superpotent topical corticosteroids or oral corticosteroids.
KeywordsHemidesmosome Immunoglobulin Basement membrane zone Autoimmune disease Vesiculobullous disease Pemphigoid
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On the Web
- N-versus-u-test: practice serration pattern analysis http://www.nversusu.umcg.nl.