Pemphigoid gestationis

What causes pemphigoid gestationis?

Pemphigoid gestationis (PG) is an autoimmune disease, which means that an affected person's immune system mistakenly reacts against the person's own tissue. Immunoglobulin type G (IgG) autoantibodies, which normally protect the body against infections, are responsible in PG. The antibody attack results in inflammation and separation of the epidermis (outer layer of skin) from the dermis (inner layer of skin), allowing fluid to build up and create the blisters associated with PG. The exact, underlying triggers that cause a woman to develop PG are still being studied.

Last updated on 05-01-20

How is pemphigoid gestationis diagnosed?

Pemphigoid gestationis shares some common features with other skin conditions of pregnancy, which can make diagnosis difficult. Diagnosis generally first requires a skin biopsy, which shows typical features of subepidermal blistering. The diagnosis may then be confirmed by direct immunofluorescence (DIF) staining of the biopsy to reveal antibodies. This is a lab technique that uses fluorescent dyes to identify antibodies bound to specific antigens. In some cases, circulating antibodies can be detected by a blood test (indirect immunofluorescence test).

Last updated on 05-01-20

What is pemphigoid gestationis?

Pemphigoid gestationis (PG) is a pregnancy-associated, autoimmune skin disorder. It usually begins abruptly during the 2nd or 3rd trimester of pregnancy, but it can begin at any time during pregnancy. Signs and symptoms often include the sudden formation of very itchy, red bumps and/or blisters on the abdomen and trunk, which may then spread to other parts of the body. Unrelenting itchiness (pruritus) often interferes with daily activities. Symptoms may improve at the end of pregnancy, but flares may occur during, or right after, delivery. While PG usually goes away on its own within weeks to months after delivery, it has been reported to persist for years in some cases. PG is caused by a woman's immune system producing autoantibodies and mistakenly attacking her own skin, but the trigger for autoantibody production is poorly understood. Treatment aims to relieve itching and prevent blister formation, and may involve the use of topical corticosteroids, oral corticosteroids, and/or oral antihistamines. The lowest effective dose of medication should be used in order to minimize the risk to the mother and fetus. The disorder may recur at a later time such as when menstruation resumes; with use of oral contraceptives; and/or during subsequent pregnancies.

Last updated on 05-01-20

Does pemphigoid gestationis recur in future pregnancies?

Yes. Approximately 95 percent of women have PG with future pregnancies and lesions may be more severe, appear earlier during the pregnancy, and last longer after delivery.

Last updated on 05-01-20

Does pemphigoid gestationis affect the fetus? If so, how?

Mothers with PG may be at an increased risk of having a baby that is small- for-gestational age or preterm. Some have suggested that mild placental insufficiency may be the cause of these risks. Sonograms of the baby during the third trimester might be appropriate and used to monitor fetal growth and development. Another risk associated with maternal PG is the placental trasnder of the PG antibody, resulting in neonatal PG; this occurs in about 5 to 10 percent of babies. The blisters resolve spontaneously without scarring over a period of weeks as the maternal PG antibodies are cleared by the baby. Babies with neonatal PG should be monitored for bacterial infections of the lesions. Early treatment can prevent progression to systemic infection.

Last updated on 05-01-20

Are there long-term effects, such as infertility, for children born to mothers with pemphigoid gestationis?

To our knowledge, there is no data on the long-term prognosis for children of mothers who had pemphigoid gestationis (PG). Hence, we are not aware of any data regarding whether PG might affect fertility in the offspring of affected women. We are only aware of information about potential effects of PG on the fetus and the newborn.

PG is associated with an increased risk of premature birth, and being small- for-gestational-age. To our knowledge, no specific congenital abnormalities have been linked to PG. While the IgG antibodies present with PG do pass through the placenta, only about 3% of newborn infants develop PG blisters. However, skin symptoms in newborns usually resolve quickly without treatment as the antibody levels decrease. If the mother was treated with large doses of cortisone, there is a possibility of neonatal adrenal insufficiency to occur.

Last updated on 05-01-20

Are there any long-term complications associated with pemphigoid gestationis?

In general, there are no long-term complications associated with PG. However, an association between PG and other autoimmune diseases like Graves' disease has been reported; therefore, it is performance of immediate and periodic screening tests of thyroid function is recommended. Some women will have persistent HG or recurrent flares lasting weeks or months following birth. Additionally, it is important to recognize that women who have had PG are at an increased risk of postpartum flare-ups, if they use oral contraceptives within 6 months of giving birth.

Last updated on 05-01-20

Who might I contact for information about potential long-term effects of pemphigoid gestationis on children?

When a condition is rare, there is often little information in the literature and it can be difficult to find someone who has seen many cases. We encourage you to view our fact sheet How to Find a Disease Specialist, which provides strategies for identifying healthcare professionals and/or researchers who have experience with a particular condition.

Last updated on 05-01-20

What are the effects of prenatal exposure to prednisone?

Taking an oral corticosteroid like prednisone long-term during pregnancy has been associated with an increased chance for delivering a baby that is premature (born before 37 weeks of pregnancy) and/or has a lower birth weight than expected.

Older studies have suggested a small increased chance of oral clefts when oral corticosteroids like prednisone are used in the first trimester. However, a number of other studies have not shown an increase in the chance for birth defects (including oral clefts) with first trimester exposure. If there is a risk from first trimester exposure to prednisone, it is thought to be very small.

There have also been concerns that some of the toxicity associated with glucocorticoids in adults might affect exposed fetuses. However, long-term follow-up of children of mothers who received prednisone as an immunosuppressant during pregnancy did not identify adverse physical, immunologic, or neurodevelopmental effects of exposure. A follow-up study in pre-pubertal children of mothers receiving prednisone during pregnancy found higher daytime cortisol concentrations in the children, but no associated adverse effects. To our knowledge, no effects on fertility in humans has been reported.

Women who are pregnant, or planning to become pregnant, should speak with their healthcare provider about their treatment plan. In many cases, the decision to begin or continue a medication during pregnancy is made on a case- by-case basis, after careful consideration of the benefits and risks.

Last updated on 05-01-20

How might pemphigoid gestationis be treated?

The goals of treatment for women with pemphigoid gestationis (PG) are to relieve itching, prevent blister formation, and treat any secondary infections. Treatment may depend on the severity in each person, and the risks and benefits of therapies need to be considered for both the mother and the fetus. In most cases, PG resolves spontaneously (on its own) within days after delivery, so treatment can usually be tapered off and stopped.

Topical corticosteroids may be used in milder cases, while oral corticosteroids are needed in more severe cases. Oral antihistamines may also be used to relieve itching. Intravenous immunoglobulin (IVIG) has also been reported to be effective. Certain immunosuppressive medications may also be effective, but their safety during pregnancy and/or breastfeeding must be considered.

Last updated on 05-01-20

Name: International Pemphigus & Pemphigoid Foundation 1331 Garden Highway, Suite 100
Sacramento, CA, 95833, United States
Phone: 916-922-1298 Toll Free: 855-473-6744 Fax : 916-922-1458 Email: info@pemphigus.org Url: http://www.pemphigus.org

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