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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.
Bullous pemphigoid (a type of pemphigoid) is an autoimmune pruritic skin disease that typically occurs in people aged over 60, that may involve the formation of blisters in the space between the epidermal and dermal skin layers.
Mucous membrane pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin. [3] It is one of the pemphigoid diseases that can result in scarring.
A Dupixent (dupilumab) pivotal study (ADEPT) in bullous pemphigoid (BP) met the primary and all key secondary endpoints evaluating its investigational use in adults with moderate-to-severe disease. In the study, five times more Dupixent patients achieved sustained disease remission compared to those on placebo.
This article provides a list of autoimmune diseases. These conditions, where the body's immune system mistakenly attacks its own cells, affect a range of organs and systems within the body. Each disorder is listed with the primary organ or body part that it affects and the associated autoantibodies that are typically found in people diagnosed ...
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.
Circulating auto-antibodies in the human body can target normal parts of the skin leading to disease. This is a list of antigens in the skin that may become targets of circulating auto-antibodies leading to the various types of pemphigoid .
The cause of the disease is a haploinsufficiency of the enzyme ATP2C1; [7] the ATP2C1 gene is located on chromosome 3, which encodes the protein hSPCA1.A mutation on one copy of the gene causes only half of this necessary protein to be made and the cells of the skin do not adhere together properly due to malformation of intercellular desmosomes, causing acantholysis, blisters and rashes.