What are the other Names for this Condition? (Also known as/Synonyms)

• Gestational Pemphigoid
• PG (Pemphigoid Gestationis)

What is Pemphigoid Gestationis? (Definition/Background Information)

• Pemphigoid Gestationis (PG) is described as a rare type of autoimmune disorder that manifests as itchy and blistering skin rashes, anytime during pregnancy
• The rashes are present on the abdomen (distended abdominal skin) and may spread to involve the trunk and arms. The blisters may break and cause superimposed infections, which may rarely lead to scarring of skin on healing
• Pemphigoid Gestationis may be observed in women with hormonal imbalances, or in those who had used oral contraceptives earlier
• There is no cure for Pemphigoid Gestationis, although the symptoms may be reduced through application of moisturizing creams and short-term steroids, when necessary
• The prognosis of Pemphigoid Gestationis is good in a majority of cases. It is known to disappear after delivery of the baby

Pemphigoid Gestationis was previously identified with herpes viral infection; hence, it used to be wrongly termed (earlier) as herpes gestationis or pemphigoid (herpes) gestationis. However, no association to infection by herpes virus is established, and the use of the term ‘herpes’ in the context of Pemphigoid Gestationis is discontinued and discouraged.

Who gets Pemphigoid Gestationis? (Age and Sex Distribution)

• Pemphigoid Gestationis may occur at any stage during pregnancy, but it is more common in the second and third trimester i.e., between 4-9 months of pregnancy
• The condition is rare and has an incident rate of 1 in 50,000 pregnancies
• Individuals of all racial and ethnic background can be affected, although Caucasians are affected more than any other races

What are the Risk Factors for Pemphigoid Gestationis? (Predisposing Factors)

The risk factors for Pemphigoid Gestationis may include:

• Use of oral contraceptives
• Estrogen and progesterone hormonal dysfunction
• ‘Small for gestational age fetus’ (meaning that the baby is not developing well in the womb) is known to be associated with the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pemphigoid Gestationis? (Etiology)

• Pemphigoid Gestationis is an autoimmune disorder that occurs during pregnancy. The condition is caused by autoantibodies that form in the body against a protein called BPAG2
• The protein is found in the basement membrane of skin. It is responsible in holding various layers of the skin together
• When autoantibodies attack these proteins, it cause an abnormal inflammatory reaction leading to fluid accumulation below the skin surface causing blister formation
• The hormone estrogen is believed to worsen the condition when it increases in the body (during pregnancy or following the use of oral contraceptive pills)
• The condition is not caused by what the expectant mother does or does not do, either prior to or during pregnancy

Pemphigoid Gestationis is non-contagious and it cannot be transmitted from one individual to another.

What are the Signs and Symptoms of Pemphigoid Gestationis?

The signs and symptoms of Pemphigoid Gestationis include:

• Presence of itchy red rashes that form into blisters anywhere on the body skin
• The itchy bumps commonly occur around the umbilicus (abdomen) and then spread to the chest, back, buttocks and arms
• The palms, soles, and oral cavity are only rarely involved. It usually does not affect the head and neck area
• These bumps or papules merge together to form plaques; some of the plaques can turn into blisters

How is Pemphigoid Gestationis Diagnosed?

A diagnosis of Pemphigoid Gestationis may involve the following:

• A thorough medical history assessment and complete physical examination
• Assessment of the presenting signs and symptoms
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Immunofluorescence studies on a skin biopsy specimen
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pemphigoid Gestationis?

The complications of Pemphigoid Gestationis are rare, but may include:

• The rashes may ulcerate and bleed
• Chronic itching may cause difficulty in sleeping
• Secondary bacterial and fungal infections: The condition may provide a suitable environment for bacteria and fungi to grow and thrive, resulting in secondary infections. This may lead to scarring of skin
• Cosmetic issues resulting in emotional stress in some individuals
• Preterm delivery, in rare cases
• The newborn child may be affected through maternal blood and symptoms of the skin condition may be observed
• Recurrence of Pemphigoid Gestationis in subsequent pregnancy

How is Pemphigoid Gestationis Treated?

It is very important to note that the condition should be carefully evaluated by a qualified healthcare provider, before starting and/or performing any treatment. This is because the treatment may have the potential to affect the developing fetus and the guidance of a healthcare expert is crucial.

There is no cure for Pemphigoid Gestationis. The treatment is usually undertaken based on the specific set of skin symptoms noted and under the recommendation of a professional healthcare provider. The treatment may include:

• Use of moisturizing creams and topical steroids
• If the symptoms are severe, then low-dose systemic steroids may be prescribed for a short duration
• In some individuals, anti-histamine therapy may be recommended, to relieve itching
• Immunosuppressants may be used to decrease the requirement of corticosteroids
• Antibiotics or antiviral medication to treat secondary infections, if needed

How can Pemphigoid Gestationis be Prevented?

Currently, there are no known methods to prevent the occurrence of Pemphigoid Gestationis. However, one may lower the risk for the skin condition by considering the following factors:

• Treating any underlying conditions early and adequately
• Careful and periodic monitoring of the pregnancy is advised and recommended
• Inform your physician if you are allergic to certain medications and foods

What is the Prognosis of Pemphigoid Gestationis? (Outcomes/Resolutions)

The prognosis of Pemphigoid Gestationis is generally good in most of the individuals.

• The symptoms either completely disappear or get much better after delivery. However, in around 80% of the women, the symptoms get worse around delivery time
• In some cases, the symptoms may occur in the newborn child. This is due to passing of autoantibodies from the mother to the infant during pregnancy. When such autoantibodies clear up in the newborn baby, typically within 3-4 months, the symptoms are known to go away
• Pemphigoid Gestationis can recur in subsequent pregnancies, in some cases

Additional and Relevant Useful Information for Pemphigoid Gestationis:

• Current studies indicate that it is safe for women with Pemphigoid Gestationis to breastfeed their children, even though they may be on prescription steroid (oral) medication. However, it is best to clarify on this with the healthcare provider before breastfeeding the child
• Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition
• The presence of dirt on the body is not a causative factor for the condition. However, it helps to be clean and hygienic, which may help the condition from getting worse