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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.
Drug-induced pemphigus: IgG: Desmoglein 3 Desmoglein 1: 130 160 Subcorneal pustular dermatosis type of IgA pemphigus: IgA1: Desmocollin 1: 110/100 Intraepidermal neutrophilic type of IgA pemphigus: IgA1: Unknown: Pemphigus erythematosus (Senear–Usher syndrome) [nb 3] IgG: Desmoglein 1: 160 Fogo selvagem: IgG: Desmoglein 1: 160 Pemphigus ...
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 vulgaris is a relatively rare disease that only affects about 1 to 5 people in 1 million in the United Kingdom, with an incidence of 1-10 cases per 1 million people across the world. There is an estimated prevalence of 14,000+ cases in the United States [ 14 ] and 42,400+ in the seven major markets (US, UK, Japan, Germany, France ...
Pemphigus foliaceus is an autoimmune blistering disease of the skin. [1] Pemphigus foliaceus causes a characteristic inflammatory attack at the subcorneal layer of epidermis, which results in skin lesions that are scaly or crusted erosions with an erythematous (red) base. [2] Mucosal involvement is absent even with widespread disease. [3]
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]
When the cornea of the eye is affected, repeated scarring may result in blindness. Brunsting–Perry cicatricial pemphigoid is a rare variant of mucous membrane pemphigoid involving the scalp and the neck without mucosal involvement. It is proposed by some authors that this be called a variant of epidermolysis bullosa acquisita. [6] [1]
IgA pemphigus is a subtype of pemphigus with two distinct forms: Subcorneal pustular dermatosis (also known as Sneddon–Wilkinson disease and pustulosis subcornealis) [ 1 ] is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils.