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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 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]
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.
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]
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]
The NIAMS is committed to pursuing new opportunities in genetics and genomics research, clinical trial design, pain, and biopsychosocial aspects of diseases in this portfolio. It is also committed to identification of risk factors for these disorders, enhancement of disease prediction, and advancement of prevention strategies.
Familial benign chronic pemphigus (familial benign pemphigus, Hailey–Hailey disease) Fanconi syndrome (familial pancytopenia, familial panmyelophthisis) Fibrodysplasia ossificans progressiva; Focal dermal hypoplasia (Goltz syndrome) Follicular atrophoderma; Franceschetti–Klein syndrome (mandibulofacial dysostosis)
Being female is the single greatest risk factor for developing autoimmune disease than any other genetic or environmental risk factor yet discovered. [ 23 ] [ 24 ] Autoimmune conditions overrepresented in women include: lupus , primary biliary cholangitis , Graves' disease , Hashimoto's thyroiditis , and multiple sclerosis , among many others.