Gestational pemphigoid
Encyclopedia
Gestational Pemphigoid or Pemphigoid Gestationis (PG) is a dermatosis of pregnancy, being an autoimmune blistering skin disease that occurs during pregnancy
, typically in the second or third trimester, and/or immediately following pregnancy. It was originally called herpes gestationis because of the blistering appearance, although it is not associated with the herpes virus.
response that causes extreme relentless itching (pruritus).
become confused and begin to increase in number. These antibodies are directed at the skin and attack the protein that binds the outer layer of skin to the body. This action causes the skin to lift up, creating blisters.
The condition involves deposition of C3 at the dermal-epidermal junction. The autoantibody is IgG.
(pruritic urticarial papules and plaques of pregnancy), especially if it occurs in a first pregnancy. PUPPP typically begins in stretch mark areas of the abdomen and usually ends within 2 weeks after delivery. PUPPP is not an autoimmune disease.
Diagnosing PG is done by biopsy
using direct immunofluorescence
(DIF); appearance; and blood studies.
; and/or topical steroids, i.e. clobetasol and betamethasone
. Suppressing the immune system with corticosteroids helps by decreasing the number of antibodies that are attacking the skin. Treating PG can be difficult and can take several months. Some cases of PG persist for many years.
There is no cure for PG. Women who have PG are considered in remission if they are no longer blistering. Remission can last indefinitely, or until a subsequent pregnancy. PG usually occurs in subsequent pregnancies; however, PG often seems more manageable because it is anticipated.
Pregnant women with PG should be monitored for conditions that may affect the fetus, including, but not limited to, low or decreasing volume of amniotic fluid
and preterm labor.
Pregnancy
Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...
, typically in the second or third trimester, and/or immediately following pregnancy. It was originally called herpes gestationis because of the blistering appearance, although it is not associated with the herpes virus.
Presentation
PG typically starts as a blistering rash in the naval area and then spreads over the entire body. It is sometimes accompanied by raised, hot, painful welts called plaques. There have been cases where only plaques occurred. PG creates a histamineHistamine
Histamine is an organic nitrogen compound involved in local immune responses as well as regulating physiological function in the gut and acting as a neurotransmitter. Histamine triggers the inflammatory response. As part of an immune response to foreign pathogens, histamine is produced by...
response that causes extreme relentless itching (pruritus).
Causes
The cause of PG is not known, although many researchers believe that the antibodies that protect the placentaPlacenta
The placenta is an organ that connects the developing fetus to the uterine wall to allow nutrient uptake, waste elimination, and gas exchange via the mother's blood supply. "True" placentas are a defining characteristic of eutherian or "placental" mammals, but are also found in some snakes and...
become confused and begin to increase in number. These antibodies are directed at the skin and attack the protein that binds the outer layer of skin to the body. This action causes the skin to lift up, creating blisters.
The condition involves deposition of C3 at the dermal-epidermal junction. The autoantibody is IgG.
Differential diagnosis
PG often is confused with PUPPPPUPPP
Pruritic urticarial papules and plaques of pregnancy , known in United Kingdom as polymorphic eruption of pregnancy , is a chronic hives-like rash that strikes some women during pregnancy. Although extremely annoying for its sufferers , it presents no long-term risk for either the mother or unborn...
(pruritic urticarial papules and plaques of pregnancy), especially if it occurs in a first pregnancy. PUPPP typically begins in stretch mark areas of the abdomen and usually ends within 2 weeks after delivery. PUPPP is not an autoimmune disease.
Diagnosing PG is done by biopsy
Biopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
using direct immunofluorescence
Immunofluorescence
Immunofluorescence is a technique used for light microscopy with a fluorescence microscope and is used primarily on biological samples. This technique uses the specificity of antibodies to their antigen to target fluorescent dyes to specific biomolecule targets within a cell, and therefore allows...
(DIF); appearance; and blood studies.
Treatment
The most accepted way to treat PG is with the use of corticosteroids, i.e. prednisonePrednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
; and/or topical steroids, i.e. clobetasol and betamethasone
Betamethasone
Betamethasone is a potent glucocorticoid steroid with anti-inflammatory and immunosuppressive properties. Unlike other drugs with these effects, betamethasone does not cause water retention. It is applied as a topical cream, ointment, foam, lotion or gel to treat itching...
. Suppressing the immune system with corticosteroids helps by decreasing the number of antibodies that are attacking the skin. Treating PG can be difficult and can take several months. Some cases of PG persist for many years.
There is no cure for PG. Women who have PG are considered in remission if they are no longer blistering. Remission can last indefinitely, or until a subsequent pregnancy. PG usually occurs in subsequent pregnancies; however, PG often seems more manageable because it is anticipated.
Pregnant women with PG should be monitored for conditions that may affect the fetus, including, but not limited to, low or decreasing volume of amniotic fluid
Amniotic fluid
Amniotic fluid or liquor amnii is the nourishing and protecting liquid contained by the amniotic sac of a pregnant woman.- Development of amniotic fluid :...
and preterm labor.