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Pemphigoid (herpes) gestationis

PEMPHIGOID (HERPES) GESTATIONIS

The aims 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 mid to late pregnancy (13 to 40 weeks gestation, known as the second and third trimesters) with an itchy rash that develops into blisters. It may recur in subsequent pregnancies and occasionally recurs in women who go on to take oral contraceptive therapy or during menstruation when their periods restart after pregnancy.

It was previously known as herpes gestationis but this name has been changed as there is no association with herpes virus (cold sore) infection.

What causes pemphigoid gestationis?

Pemphigoid gestationis is an auto-immune blistering disease that affects pregnant women. This means that the mother’s immune system starts reacting against her own skin causing the skin to split and form blisters.  Current knowledge suggests that some of the placenta tissue enters the mother’s blood stream and causes her immune system to activate and attacks the skin causing blisters.

Female hormones (particularly oestrogen) are thought to aggravate the condition and this may be why the condition presents during pregnancy when oestrogen levels rise and recurs when oral contraceptives containing oestrogen are taken.

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 and what does it look like?

Itching is common and often starts around the umbilicus. It starts with urticarial 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 may also occur on palms and soles. The blisters contain clear fluid though occasionally this can be blood-stained. The blisters usually heal without scarring.

How will pemphigoid gestationis be diagnosed?

Usually the appearance and behaviour of the rash is very typical and easily diagnosed by a dermatologist. However, before the blisters appear the rash can look similar to other skin diseases. Your doctor may take a sample of skin and a blood test to look for the antibodies that will confirm the diagnosis.

Can pemphigoid gestationis be cured?

No, but it can be suppressed with treatment. Symptoms often improve towards the end of pregnancy but 80% of women will experience a flare of the rash at the time of delivery. Symptoms usually resolve days or weeks after giving birth, but in some women the disease can remain active for months and may require continued treatment. Restarting periods, use of oral contraceptive therapy and further pregnancies can all cause flare-ups of the condition.

How can pemphigoid gestationis be treated?

The primary aim of treatment is to relieve itching, prevent blister formation and treat any secondary infection.

Topical steroid creams can be used in mild cases if only a limited area of skin is affected. If the rash is quite extensive a stronger steroid cream may be recommended.

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.

Oral antihistamines (those suitable for use during pregnancy) can be used to relieve itching.

Steroid tablets are usually needed to control the blisters and itching. The dose of steroid tablets may need to be increased at the end of pregnancy to prevent the disease flaring after delivery.

Occasionally other medication may be used if the condition is severe or does not respond to treatment with steroids.

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 with the rash lasting up to 6 weeks until the mother’s antibodies are cleared from the baby’s system. Usually only mild treatment is required such as steroid or antibiotic creams and emollients.

The baby is at increased risk of premature delivery and may be relatively small as compared to a normal full term baby. It is important that the obstetrician, and midwives monitor the pregnancy closely.

Is normal delivery possible?

Yes, Caesarean section is not routinely necessary for this condition unless there are other indications. Blistering of the vulva and vagina occurs in 20% of affected women.

Are the treatments safe for the baby and mother?

Taking steroids by mouth means an increased risk in the mother of developing diabetes (raised sugar levels) and hypertension (raised blood pressure). Regular checks of blood pressure and urine and ultrasound scans are important. Women who have been taking steroid treatment for a prolonged period should not stop them suddenly but should follow their doctor’s advice and reduce treatment gradually.

Can women with pemphigoid gestationis still breastfeed?

Yes. It is safe to breastfeed even while taking steroid tablets as only very small amounts of steroid get into breast milk. There is some evidence that breastfeeding may improve the condition.

Where can I get more information about pemphigoid gestationis?

Web links to detailed leaflets:

www.dermnetnz.org/immune/pemphigoid-gestationis.html (includes photographs)

For details of source materials used please contact the Clinical Standards Unit (clinicalstandards@bad.org.uk).

This leaflet aims to provide accurate information about the subject and is a consensus of the views held by representatives of the British Association of Dermatologists: its contents, however, may occasionally differ from the advice given to you by your doctor.

This leaflet has been assessed for readability by the British Association of Dermatologists’ Patient Information Lay Review Panel

BRITISH ASSOCIATION OF DERMATOLOGISTS PATIENT INFORMATION LEAFLET PRODUCED SEPTEMBER 2007 UPDATED NOVEMBER 2010, JANUARY 2014 REVIEW DATE JANUARY 2017

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