The aim of this leaflet
This leaflet is designed to tell you more about Pemphigoid Gestationis. It tells you what the condition is, what causes it, what can be done about it and where to find out more about it.

What is Pemphigoid Gestationis?
Pemphigoid Gestationis is a rare skin blistering disorder that occurs in women of childbearing age. It usually presents in pregnancy but can also recur in women who subsequently take oral contraceptive therapy or with menstruation.
It usually starts with an itchy rash that develops into blisters. It is commonest during mid to late pregnancy (the second and third trimesters). It was previously known as herpes gestationis but this name has been abandoned as there is no association with herpes virus infection.

What causes Pemphigoid Gestationis?
Pemphigoid Gestationis is an auto-immune blistering disease which basically means that an individual’s immune system starts reacting against his or her own skin causing skin splitting and blistering.
Female hormones (particularly oestrogen) are thought to aggravate the reaction and this may be  why it often presents during pregnancy when oestrogen levels rise. Pemphigoid Gestationis may recur in subsequent pregnancies and also may recur in some women who go on to take oral contraceptive therapy or during menstruation when their periods re-start following pregnancy.

Does Pemphigoid Gestationis run in families?
No but there is a link with other auto-immune diseases (which may run in families) such as thyroid disease and pernicious anaemia.

What are the symptoms of Pemphigoid Gestationis?
Itching is common and often starts around the umbilicus (belly button) during mid to late pregnancy (13 to 40 weeks gestation). Itching may be followed by a rash with large red inflamed areas of skin and then later blisters can develop.

What does Pemphigoid Gestationis look like?
There is often an urticarial rash with wheals (like hives from nettles) and large raised red patches (plaques) commonly occurring on the trunk, back, buttocks and limbs. The face, scalp and mucous membranes (mouth and genital area) are usually not involved.  Large tense blisters then occur on the red patches within 1-2 weeks, and are also seen on palms and soles. The blisters usually contain clear fluid or occasionally blood-stained fluid. There is usually no scarring when the blisters heal.

How will Pemphigoid Gestationis be diagnosed?
Diagnosis requires a skin sample (biopsy) and/or a blood for laboratory tests. Usually the appearance and behaviour of the rash is very typical but in early disease without blisters the rash can look reminiscent of other skin diseases such as eczema, polymorphic eruption of pregnancy (PEP), or a drug eruption. Special tests should  be done on blood and a skin biopsy (immunofluorescence) to detect antibodies which confirms the diagnosis.

Can Pemphigoid Gestationis be cured?
No. It can be suppressed with treatment, and symptoms often improve towards the end of pregnancy but then 80% of women will experience a flare of the rash around the time of delivery. In most cases symptoms resolve days or weeks after giving birth, but in some women the disease can remain active for months or years and may require continued treatment. Restarting periods, use of oral contraceptive therapy and further pregnancies can all cause flare-ups of the disease.

How can Pemphigoid Gestationis be treated?
The primary aim of treatment is to relieve itching, prevent blister formation and treat any secondary infection. It is also important during pregnancy to use treatments that are as safe as possible for both mother and baby.
Topical steroid creams can be used in mild cases if only a limited area of skin is affected. Even if the rash is quite extensive a trial of a strong steroid cream may be worthwhile before steroid tablets are given, as creams are less likely to cause side effects.
Oral antihistamines (only those suitable for use during pregnancy) can be used to relieve itching.
Treatment for more severe disease (with blistering) is usually with high doses of steroid tablets to get the disease under control rapidly. This needs careful monitoring and should involve the obstetricians, to look at both the health of the mother and baby. The dose of steroid tablets may need to be increased at the end of pregnancy to prevent the disease flaring after delivery.
Blisters may be burst (with a sterile needle) to offer relief from discomfort and dressings can be applied to weepy or raw areas of skin. Emollient creams or ointments can also be applied to reduce itching and soothe sore areas.
Other drugs may be used in more severe cases or in women who experience severe disease following delivery.

Will the baby be affected?
Occasionally the baby will develop a blistering rash following delivery due to transfer of the mother’s antibodies across the placenta. This only occurs in 5-10% of cases and the rash is usually only temporary lasting up to 6 weeks until the mother’s antibodies are cleared. Usually mild treatment only is required such as antibiotic creams and dressings.
The baby is at increased risk of premature delivery and may be relatively small for dates. With this in mind it is important that the obstetrician and dermatologist monitor the pregnancy closely with careful observation of the baby’s size and growth, particularly if the mother is taking steroid tablets.

Is normal delivery possible?
Yes and Caesarean section is not recommended for this condition unless there are other indications. Blistering can develop at the site of the scar and may require treatment. Blistering of the vulva and vagina can occur but only in about 20% of cases.

Are the treatments safe for the baby and mother?
With oral steroids there is an increased risk in the mother of developing diabetes (raised sugar levels) and hypertension (raised blood pressure). Careful observation of blood pressure and urine checks are therefore essential at antenatal clinic, while ultrasound scans can look for any changes in the baby’s growth. Women who have been taking steroid treatment for a prolonged period may not be able to stop the drugs immediately and should see a doctor for advice about the best way to reduce treatment.

Can women with Pemphigoid Gestationis still breastfeed?
Yes. Even while taking steroid tablets women should still be encouraged to breastfeed as only negligible amounts of steroid get into breast milk. There is some evidence that breastfeeding may improve the disease activity in PG.

Is any special monitoring required?
Yes, regular review at an antenatal clinic is even more important if the mother is taking steroid tablets orally and blood tests, urine tests, blood pressure checks with ultrasound scans are all extremely important to monitor the mother and baby’s wellbeing.

Where can I get more information about Pemphigoid Gestationis?
Link to on-line support group:
http://health.groups.yahoo.com/group/PemGest/

Reference used to prepare the text:
British Association of Dermatologists patient information leaflet for Bullous Pemphigoid (August 2004)

Web links to detailed leaflets:
www.dermnetnz.org/immune/pemphigoid-gestationis.html
www.emedicine.com/derm/topic178.htm

This leaflet has been prepared by the EADV task force “skin disease in pregnancy”, it does not necessarily reflect the official opinion of the EADV. July 2007