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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 tissue is skin from a patient with Pemphigus vulgaris. Note the intercellular IgG deposits in the epidermis and the early intraepidermal vesicle caused by acantholysis. Pemphigus ( / ˈ p ɛ m f ɪ ɡ ə s / or / p ɛ m ˈ f aɪ ɡ ə s / ) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes . [ 1 ]
It is also associated with pemphigus vulgaris and pemphigus foliaceus. [6] [7] It is useful in differentiating between the diagnosis of pemphigus vulgaris or mucous membrane pemphigoid (where the sign is present) and bullous pemphigoid (where it is absent). The Nikolsky sign is dislodgement of intact superficial epidermis by a shearing force ...
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 ...
Pemphigus vulgaris; Cytomegalovirus; Arnault Tzanck did the first cytological examinations in order to diagnose skin diseases. [3] To diagnose pemphigus, he identified acantholytic cells, and to diagnose of herpetic infections he identified multinucleated giant cells and acantholytic cells.
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.
This protein, along with Desmoglein-1, has been identified as the autoantigen of the autoimmune skin blistering disease pemphigus vulgaris. [8] The mucosal dominant form of pemphigus vulgaris only involves antibodies against Desmoglein-3 and causes mucosal erosions, but no skin lesions. [7]