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.
Pemphigoid variant | Antibody isotype | Antigen | Molecular weight (KDa) |
---|---|---|---|
Bullous pemphigoid | IgG, IgE [1] | NC16A terminal of BPAG2 BPAG1 | 180 230 |
Gestational pemphigoid | IgG1 | NC16A terminal of BPAG2 BPAG1 | 180 230 |
Cicatricial pemphigoid | C-terminal of BPAG2 | 180 | |
Ocular cicatricial pemphigoid | Integrin beta-4 subunit | 200 | |
Antiepiligrin cicatricial pemphigoid [nb 1] | Laminin 5 (Epiligrin, Laminin 332) | ||
Linear IgA bullous dermatosis | LAD1 portion of BPAG2 Collagen type VII | 97 290/145 | |
Protein 105 pemphigoid | Protein in lower lamina lucida | 105 |
Of note, there are also several other diseases that are caused by auto-antibodies that target the same anatomic area of the skin which is termed the basement membrane zone. These diseases include:
Disease | Antibody isotype | Antigen | Molecular weight (KDa) |
---|---|---|---|
Epidermolysis bullosa acquisita | Collagen type VII | 290/145 | |
Bullous lupus erythematosus | Collagen type VII | 290/145 | |
Lichen planus pemphigoides [nb 2] | BPAG2 | 180 |
Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, and is only named that because it looks like one. It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale, commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. Although there is a broad clinical range of LP manifestations, the skin and oral cavity remain as the major sites of involvement. The cause is unknown, but it is thought to be the result of an autoimmune process with an unknown initial trigger. There is no cure, but many different medications and procedures have been used in efforts to control the symptoms.
Pemphigus is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. The name is derived from the Greek root pemphix, meaning "pustule".
Bullous pemphigoid is an autoimmune pruritic skin disease preferentially in older people, aged over 60, that may involve the formation of blisters (bullae) in the space between the epidermal and dermal skin layers. The disorder is a type of pemphigoid. It is classified as a type II hypersensitivity reaction, with the formation of anti-hemidesmosome antibodies.
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, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.
Gestational pemphigoid (GP) is an autoimmune blistering skin disease of pregnancy, typically occurring in the second or third trimester. It was originally called herpes gestationis because of the blistering appearance, although it is not associated with the herpes virus. It is one of the pemphigoid (pemphigus-like) diseases.
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.
Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected. It was first described by Tadeusz Chorzelski in 1979 and may be divided into two types:
Mucous membrane pemphigoid is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of the mucous membranes and skin. It is one of the pemphigoid diseases that can result in scarring.