Gestational pemphigoid

Diagnosis of GP becomes clear when skin lesions progress to tense blisters during the second or third trimester. The face and mucous membranes are usually spared. GP typically starts as a blistering rash in the navel area and then spreads over the entire body. It is sometimes accompanied by raised, hot, painful welts called plaques. After one to two weeks, large, tense blisters typically develop on the red plaques, containing clear or blood-stained fluid.[4] GP creates a histamine response that causes extreme relentless itching (pruritus). GP is characterized by flaring and remission during the gestational and sometimes post partum period. Usually after delivery, lesions will heal within months, but may reoccur during menstruation.

Pathogenically, it is a type II hypersensitivity reaction where circulating complement-fixing IgG antibodies bind to an antigen (a 180-kDa protein, BP-180) in the hemidesmosomes (attach basal cells of epidermis to the basal lamina and hence to dermis) of the dermoepidermal junction, leading to blister formation as loss of hemidesmosomes causes the epidermis to separate from dermis. The immune response is even more highly restricted to the NC16A domain. The primary site of autoimmunity seems not to be the skin, but the placenta, as antibodies bind not only to the basement membrane zone of the epidermis, but also to that of chorionic and amniotic epithelia. Aberrant expression of MHC class II molecules on the chorionic villi suggests an allogenic immune reaction to a placental matrix antigen, thought to be of paternal origin. Recently, both IgA22 and IgE24 antibodies to either BP180 or BP230 have also been detected in pemphigoid gestationis.

The most accepted way to treat GP is with the use of corticosteroids,[8] i.e. prednisone; and/or topical steroids, i.e. clobetasol and betamethasone. Suppressing the immune system with corticosteroids helps by decreasing the number of antibodies attacking the skin. Treating GP can be difficult and can take several months. Some cases of GP persist for many years. In the post partum period, if necessary, the full range of immunosuppressive treatment may be administered for cases unresponsive to corticosteroid treatments, such as tetracyclines, nicotinamide, cyclophosphamide, ciclosporin, goserelin, azathioprine, dapsone, rituximumab, or plasmaphoresis, or intravenous immunoglobulin may sometimes be considered when the symptoms are severe.

There is no cure for GP. Women who have GP are considered in remission if they are no longer blistering. Remission can last indefinitely, or until a subsequent pregnancy. GP usually occurs in subsequent pregnancies; however, it often seems more manageable because it is anticipated.

• Bullous pemphigoid
• Cicatricial pemphigoid
• List of target antigens in pemphigoid
• List of immunofluorescence findings for autoimmune bullous conditions