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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.
Pemphigus vulgaris (PV - ICD-10 L10.0) is the most common form of the disorder and occurs when antibodies attack desmoglein 3. Sores often originate in the mouth, making eating difficult and uncomfortable. Although PV may occur at any age, it is most common among people between 40 and 60. It is more frequent among Ashkenazi Jews.
Blistering diseases such as pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune diseases in which auto-antibodies target desmogleins. PV is caused by circulating autoantibodies that target Dsg3 (Desmoglein 3) and sometimes Dsg1. PV is manifested by suprabasal acantholysis, or blisters in the mucous membrane and blisters in the ...
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: Skin and mucous membranes Anti-desmoglein 3, Anti-desmoglein 1 Confirmed 1-5 per 100,000 [21] Scleroderma (systemic sclerosis) Skin, organs ANA, anti-Scl-70, anti-centromere Confirmed 240 per 1,000,000 [22] Vitiligo: Skin Various, not specific Confirmed 1% [23]
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 ...
Pemphigus vegetans is a localized form of pemphigus vulgaris. [1] in which there is a localized vegetating papillomatous response. The eroded areas do not heal like usual but form papillomatous growth and vegetation. Accounts for 1-2% of pemphigus cases and is a relatively benign variant of pemphigus vulgaris. Two forms are recognized:
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 ...