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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.
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]
An ocular manifestation of a systemic disease is an eye condition that directly or indirectly results from a disease process in another part of the body. There are many diseases known to cause ocular or visual changes.
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 .
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]
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]
Christus Dubuis Hospital - Hot Springs, Arkansas; Conway Regional Medical Center - Conway, Arkansas; Conway Regional Rehabilitation Hospital - Conway, Arkansas; Cornerstone Specialty Hospital - Little Rock, Arkansas; Crossridge Community Hospital - Wynne, Arkansas; Dallas County Medical Center - Fordyce, Arkansas; De Queen Medical Center - De ...
Pemphigus erythematosus (Senear-Usher Syndrome) is a rare form of pemphigus with features of pemphigus foliaceus and lupus erythematosus. [ 1 ] [ 2 ] It was first described by Francis Senear and Barney Usher at the University of Illinois College of Medicine in 1926. [ 3 ]