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Pemphigoid gestationis


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Question Question


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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?

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The following information may help to address your question:

  • What is pemphigoid gestationis?
  • What are the signs and symptoms of pemphigoid gestationis?
  • What causes pemphigoid gestationis?
  • How is pemphigoid gestationis diagnosed?
  • How might pemphigoid gestationis be treated?
  • Does pemphigoid gestationis affect the fetus? If so, how?
  • Are there any long-term complications associated with pemphigoid gestationis?
  • Does pemphigoid gestationis recur in future pregnancies?

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.[1] 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.[1] 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.[1] Treatment aims to relieve itching and prevent blister formation, and may involve the use of topical corticosteroids, oral corticosteroids, and/or oral antihistamines.[2] The lowest effective dose of medication should be used in order to minimize the risk to the mother and fetus.[3] The disorder may recur at a later time such as when menstruation resumes; with use of oral contraceptives; and/or during subsequent pregnancies.[1][2]
Last updated: 1/14/2019

What are the signs and symptoms of pemphigoid gestationis?

In most women with pemphigoid gestationis (PG), the condition begins abruptly as an extremely itchy, hive-like rash during mid to late pregnancy (during the 2nd or 3rd trimester). It often begins with red bumps around the abdomen and trunk, and then spreads to other parts of the body within days to weeks. Large, fluid-filled blisters may form on the affected areas of skin. Some people with PG do not develop blisters, but instead have large, raised patches (plaques).[1][2]

Symptoms may improve or go away on their own towards the end of the pregnancy. However, most women experience a "flare" around the time of delivery. In most cases, symptoms go away again days after giving birth, but in some women the condition persists for weeks, months, or even years. The condition may occur again when menstruation resumes, with the use of oral contraceptives, or during future pregnancies.[1][2]

Some babies of women with PG grow less than expected during pregnancy (small for gestational age) and/or are born before their due date (prematurely).[1][3] While not common, some babies of women with PG are born with a rash similar to that seen in women with PG, but it typically goes away without treatment within a few weeks.[3]
Last updated: 1/14/2019

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.[4] 
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.[4]
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.[4]
Last updated: 8/11/2010

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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References References


  1. Anatoli Freiman. Pemphigoid Gestationis. Medscape Reference. August 17, 2015; http://emedicine.medscape.com/article/1063499-overview.
  2. Ngan V. Pemphigoid gestationis. DermNet New Zealand. 2003; http://www.dermnetnz.org/immune/pemphigoid-gestationis.html.
  3. Dulay AT. Pemphigoid Gestationis. MerckManuals. March, 2018; https://www.merckmanuals.com/home/women-s-health-issues/complications-of-pregnancy/pemphigoid-gestationis.
  4. Specific Dermatoses of Pregnancy. In: Gabbe SG, Niebyl JR, Simpson JL. Gabbe: Obstetrics: Normal and Problem Pregnancies, 5th ed. Philadelphia: Churchill Livingstone Elsevier; 2007;
  5. Flangini Cobo M, Giuli Santi C, Wakisaka Maruta C, Aoki V. Pemphigoid Gestationis: Clinical and Laboratory Evaluation. Clinics. 2009;

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