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The bullae are formed by an immune reaction, initiated by the formation of IgG [citation needed] autoantibodies targeting dystonin, also called bullous pemphigoid antigen 1, [6] and/or type XVII collagen, also called bullous pemphigoid antigen 2, [7] which is a component of hemidesmosomes. A different form of dystonin is associated with ...
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.
Pemphigoid is a group of rare autoimmune blistering diseases of the skin and mucous membranes.As its name indicates, pemphigoid is similar in general appearance to pemphigus, [1] however unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.
Dupixent is the first and only biologic to achieve significant improvements in disease remission and symptoms in bullous pemphigoid positive pivotal study. Study met the primary and all key secondary endpoints in adults with moderate-to-severe disease; five times more patients achieved sustained disease remission with Dupixent than placebo
Intraorally, it resembles the more common diseases lichen planus and mucous membrane pemphigoid. [13] Definitive diagnosis requires examination of a skin or mucous membrane biopsy by a dermatopathologist or oral pathologist. The skin biopsy is taken from the edge of a blister, prepared for histopathology and examined with a microscope.
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]
The disease is named because it is a porphyria that often presents with skin manifestations later in life. The disorder results from low levels of the enzyme responsible for the fifth step in heme production. Heme is a vital molecule for all of the body's organs. It is a component of hemoglobin, the molecule that carries oxygen in the blood.
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]