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Epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid

Kristin M Leiferman, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD


Bullous pemphigoid and mucous membrane pemphigoid (MMP) are uncommon autoimmune subepithelial blistering diseases that most frequently arise in older adults and are characterized by the presence of cutaneous bullae and erosive mucosal lesions. Significant progress has been made in understanding the pathogenesis of these diseases. Multiple events, including the binding of immunoglobulins to basement membrane zone components, the subsequent activation of complement, and the migration of inflammatory cells into the subepithelial tissue, likely contribute to the clinical manifestations of bullous pemphigoid and MMP.

The epidemiology and pathogenesis of bullous pemphigoid and MMP will be reviewed here. The clinical features, diagnosis, and treatment of these disorders and greater detail on the ocular form of MMP (ocular cicatricial pemphigoid) are available separately. (See "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid" and "Management and prognosis of bullous pemphigoid" and "Management of mucous membrane pemphigoid" and "Ocular cicatricial pemphigoid".)


Pemphigoid disorders, which include bullous pemphigoid, MMP, anti-laminin 332 pemphigoid (also known as anti-epiligrin cicatricial pemphigoid), pemphigoid gestationis, Brunsting-Perry pemphigoid, and anti-laminin gamma-1 (anti-p200) pemphigoid, are characterized clinically by the presence of inflammatory, blistering, and/or erosive mucocutaneous lesions and immunohistopathologically by subepithelial cleavage and immunoglobulin G (IgG) and/or complement deposits in a linear pattern at the epidermal basement membrane zone (picture 1A-B). The location of blistering and immunoglobulin deposition distinguishes pemphigoid disorders from pemphigus. In pemphigus, blister formation and antibody deposition occur within the epidermis/epithelium (picture 2A-B) [1]. (See "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid" and "Pathogenesis, clinical manifestations, and diagnosis of pemphigus".)

MMP is not a single disease, rather it represents a group of heterogeneous, chronic subepithelial blistering diseases that primarily affect mucosal surfaces [2]. Historically, because of the scarring sequelae that may develop clinically, the term cicatricial pemphigoid was ascribed to diseases now categorized as MMP.

Some authors also consider mucosal-predominant forms of linear IgA bullous dermatosis and epidermolysis bullosa acquisita as forms of MMP [2]. However, our preference is to consider these diagnoses as distinct disease entities. (See "Linear IgA bullous dermatosis" and "Epidermolysis bullosa acquisita".)

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Literature review current through: Nov 2017. | This topic last updated: Feb 07, 2017.
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