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Pathogenesis, clinical manifestations, and diagnosis of pemphigus

Michael Hertl, MD
Cassian Sitaru, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD


Pemphigus is defined as a group of life-threatening blistering disorders characterized by acantholysis (loss of keratinocyte to keratinocyte adhesion) that results in the formation of intraepithelial blisters in mucous membranes and skin [1]. The process of acantholysis is induced by the binding of circulating autoantibodies to intercellular adhesion molecules [1-3]. Patients with pemphigus develop mucosal erosions and/or flaccid bullae, erosions, or pustules on skin.

The four major entities of the pemphigus group include pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus. The different forms of pemphigus are distinguished by their clinical features, associated autoantigens, and laboratory findings.

The pathogenesis, clinical features, and diagnosis of pemphigus will be discussed here. The management of pemphigus and greater detail on paraneoplastic pemphigus are reviewed separately. (See "Initial management of pemphigus vulgaris and pemphigus foliaceus" and "Management of refractory pemphigus vulgaris and pemphigus foliaceus" and "Paraneoplastic pemphigus".)


Common features of the major types of pemphigus are reviewed briefly below. A broader summary of the clinical and laboratory features of pemphigus is provided in the table (table 1).

Pemphigus vulgaris


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