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Diseases

Genetic and Rare Diseases Information Center (GARD)

Pemphigoid gestationis


Other Names for this Disease
  • Gestational pemphigoid
  • Herpes gestationis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My daughter has been diagnosed with pemphigoid gestationis 5 weeks after giving birth.  Do you have any information that can provide information on this rare autoimmune disease? Are there any clinical studies currently being performed?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is pemphigoid gestationis (PG)?

Pemphigoid gestationis (PG) is a pregnancy-associated autoimmune skin disorder. It usually begins abruptly during the 2nd or 3rd trimester of pregnancy, but can occur any time during pregnancy. Signs and symptoms often include the formation of very itchy, red bumps and/or blisters on the abdomen and trunk, but other parts of the body can be affected. Unrelenting itchiness (pruritus) often interferes with daily activities.[1] Symptoms may improve at the end of pregnancy, but flares may occur during, or right after, delivery. While it usually goes away on its own within weeks to months after delivery, it may recur during menses; with use of oral contraceptives; and/or with subsequent pregnancies.[1] PG is caused by a woman's immune system mistakenly attacking her own skin, but the exact reasons why this occurs is poorly understood. Treatment depends on severity and may include topical corticosteroids; oral corticosteroids, and/or oral antihistamines to relieve itching.[2]
Last updated: 5/3/2016

What are the signs and symptoms of pemphigoid gestationis?

In most women with pemphigoid gestationis (PG), the condition begins as an extremely itchy, hive-like rash during mid to late pregnancy. It often begins with red bumps around the belly button, but spreads to other parts of the body within days to weeks. The face, scalp, palms, soles and mucous membranes are usually not affected. After about 2 to 4 weeks, large, fluid-filled blisters form on the affected areas. Some people with PG don't develop blisters but instead have large, raised patches (plaques).

Symptoms may improve or go away on their own towards the end of the pregnancy; however, up to 80% of women experience a "flare" around the time of delivery. In most cases, symptoms again resolve days after giving birth, but in some women the condition persists for months or years. Flares may occur when menstruation resumes; with the use of oral contraceptives; or during subsequent pregnancies.[2]
Last updated: 5/3/2016

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.[2] The exact, underlying triggers that cause a woman to develop PG are still being studied.
Last updated: 5/3/2016

How is pemphigoid gestationis diagnosed?

Pemphigoid gestationis shares some common features with other skin conditions of pregnancy, which can make diagnosis difficult.[1] 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.[2] In some cases, circulating antibodies can be detected by a blood test (indirect immunofluorescence test).[2]
Last updated: 5/3/2016

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.[1][2] 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.[1] In most cases, PG resolves spontaneously (on its own) within days after delivery, so treatment can usually be tapered off and stopped.[2]

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.[2]
Last updated: 5/3/2016

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.[3] 
Last updated: 8/11/2010

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.[3]
Last updated: 8/11/2010

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.[3]
Last updated: 8/11/2010

References
  • Anatoli Freiman. Pemphigoid Gestationis. Medscape Reference. August 17, 2015; http://emedicine.medscape.com/article/1063499-overview.
  • Vanessa Ngan. Pemphigoid gestationis. DermNet NZ. April 25, 2016; http://www.dermnetnz.org/immune/pemphigoid-gestationis.html.
  • Specific Dermatoses of Pregnancy. In: Gabbe SG, Niebyl JR, Simpson JL. Gabbe: Obstetrics: Normal and Problem Pregnancies, 5th ed. Philadelphia: Churchill Livingstone Elsevier; 2007;
  • Flangini Cobo M, Giuli Santi C, Wakisaka Maruta C, Aoki V. Pemphigoid Gestationis: Clinical and Laboratory Evaluation. Clinics. 2009;
Other Names for this Disease
  • Gestational pemphigoid
  • Herpes gestationis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.