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Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus.Pemphigus was derived from the Greek word pemphix, meaning blister. [1] It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other.
The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus) vary in severity. Skin lesions caused by pemphigus can lead to fatal infections, so treatment is extremely important.
Paraneoplastic pemphigus (PNP) is an autoimmune disorder stemming from an underlying tumor. It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes .
Pemphigus erythematosus (Senear–Usher syndrome) Pemphigus foliaceus; Pemphigus herpetiformis (acantholytic herpetiform dermatitis, herpetiform pemphigus, mixed bullous disease, pemphigus controlled by sulfapyridine) Pemphigoid nodularis; Pemphigus vegetans; Pemphigus vegetans of Hallopeau; Pemphigus vegetans of Neumann; Pemphigus vulgaris ...
Mucosal pemphigus vulgaris: IgG: Desmoglein 3: 130 Mucocutaneous pemphigus vulgaris: IgG: Desmoglein 3 Desmoglein 1: 130 160 Pemphigus foliaceus: IgG: Desmoglein 1: 160 Paraneoplastic pemphigus: IgG: Desmoglein 3 Desmoglein 1 BPAG1 [nb 1] Plectin Desmoplakin I [nb 2] Desmoplakin II Envoplakin Periplakin Alpha-2-macroglobulin-like-1: 130 160 230 ...
Hailey–Hailey disease (HHD), or familial benign chronic pemphigus [1]: 559 or familial benign pemphigus, [2]: 622 was originally described by the Hailey brothers (Hugh Edward and William Howard) in 1939. [3] [4] It is a genetic disorder that causes blisters to form on the skin.
Substrates used in indirect immunofluorescence studies for the different pemphigus variants Pemphigus variant Substrate Pemphigus foliaceous: Guinea pig esophagus Pemphigus vulgaris: Monkey esophagus IgA pemphigus: Cultured skin Paraneoplastic pemphigus: Rat bladder
[2] [3] It is seen along with Nikolsky's sign, both used to assess the severity of some blistering diseases such as pemphigus vulgaris and severe bullous drug reactions. [ 4 ] This sign is named for the Danish physician Gustav Asboe-Hansen (1917–1989), who first described it in 1960.