Management of bullous pemphigoid and mucous membrane pemphigoid

Abstract

Bullous pemphigoid (BP) is the most common blistering autoimmune dermatosis and, as an age-associated disease, is also the bullous dermatosis with the highest increase in incidence in recent years due to demographic developments. Mucous membrane pemphigoid (MMP), which is less common, is aclinically and immunopathologically heterogeneous blistering autoimmune dermatosis characterized by blisters and erosions on mucous membranes. This work summarizes the manifold clinical characteristics of both diseases and provides an up-to-date overview of diagnostics and therapy of BP and SHP. Aselective literature search was carried out. While eczematous, urticarial, or bullous lesions on the integument are typical for BP, MMP is characterized by pronounced, erosive mucosal changes orally, ocularly, genitoanally, tracheally or esophageally and, at most, only minor skin involvement. For diagnosis, the combination of histology, direct immunofluorescence microscopy, and serological detection of autoantibodies against the hemidesmosomal proteins BP180 and BP230 and, in MMP, also against laminin 332 is important. New pathophysiological findings and therapeutics have recently significantly expanded the range of treatment options. Due to the comorbidities of the usually elderly BP patients and the often multiple affected mucous membranes in MMP, with the risk of developing irreversible complications such as strictures and synechiae, interdisciplinary diagnosis and therapy is often required.