30 - Pemphigoid Group

Abstract

Bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), and epidermolysis bullosa acquisita (EBA) are autoimmune blistering diseases of the skin and mucosae that are associated with autoantibodies directed against components of the dermal–epidermal or epithelial–stromal junction of stratified epithelia. These diseases exhibit clinical overlap. BP is often associated with generalized excoriated, eczematous, urticarial and/or bullous lesions, while MMP is characterized by blistering and scarring primarily of the external mucosal surfaces. Finally, EBA presents as either a mechanobullous disorder or an inflammatory BP-like disease, but transitional forms also exist. Their precise diagnosis relies on direct immunofluorescence microscopy as well as on the characterization of the specificity of the patient’s autoantibodies. Because of the significant morbidity and specific disease sequelae, proper evaluation and patient management are essential. Topical and systemic corticosteroids represent the mainstay of therapy; immunosuppressive medications are commonly used as steroid-sparing agents and novel biological therapies, including rituximab, dupilumab and omalizumab, may be employed according to the clinical features and course, response or contraindications to standard therapies.