Pemphigoid is a rare type of skin disease with an autoimmune cause. Its symptoms are rashes and blisters all over the limbs or the abdomen, due to an error of the body's immune system. The body mistakenly identifies skin cells as aggressors and sends antibodies to attack them, which starts a chain of events that ends with one layer of skin cells being separated from the others. The condition usually affects elder people but can appear in any age, including in childhood. It can also start during pregnancy and evidence suggests the disease might also be triggered by some types of treatment.

Besides the skin, pemphigoid can make blisters appear on the mucous membranes. These are sensitive areas that produce mucus, which is used as a protective layer. Mucous membranes vulnerable to pemphigoid are the ones in the mouth, eyes and nose or on the genital organs. Some women can also develop this condition while they are pregnant.

Like most autoimmune diseases, no cure for pemphigoid is known. However, doctors have a number of treatments available to reduce the symptoms.

Types of pemphigoid

Regardless of the variety, pemphigoid has one single cause: healthy tissues are attacked by the immune system by mistake. The visible result is the appearance of rashes and blisters full of fluid. Based on the location and timing of the blisters, scientists have classified the disease into several types.

Bullous pemphigoid

Bullous pemphigoid causes typical blisters to appear, which are full of either a clear fluid or mixed with blood. The affected area is extremely itchy and covered with inflamed patches of skin with a raised profile, as well as blisters. The blisters are quite resilient and don't burst easily. Their size is variable, from a few millimetres to several centimetres. Common areas affected by bullous pemphigoid include the groin, armpits, inner thighs, lower torso, palms or soles.

Sometimes the skin around blisters is healthy but it can also be red and inflamed. The blisters rarely leave a scar after they heal, even if they can cause serious itching and pain.

This type of pemphigoid can last for years and it is a cyclical disease. Periods without any symptoms can be followed by strong outbursts, when a large number of blisters pop up.

Cicatricial pemphigoid

Cicatricial pemphigoid is a specific type of pemphigoid that only affects the mucous membranes. Usually, the condition starts in one mucous area and can expand to the others if ignored. Possible vulnerable zones are the mouth, eyes, throat, nose and genitals.

Pemphigoid gestationis

Pemphigoid gestationis refers to the disease that starts during pregnancy or immediately after it. Even if it has no relation to the herpes virus, it is sometimes called herpes gestationis because the symptoms look quite similar. The condition can start at any time during pregnancy and up to six weeks after birth but most cases begin in the second or third trimester. It mostly affects the abdomen and the limbs.

It all starts with papules, which are typical sores found around the bellybutton and elsewhere on the abdomen. The affected area then expands and starts to cover the limbs and the trunk.

After some time, usually a few weeks, blisters appear. These are often found in a circle, surrounding areas with papules. If the blisters don't get infected, scars are quite rare. Mothers can pass the disease to their child in the womb but this is a rare situation, in under 5% of recorded cases.

Pemphigoid gestationis can appear in the last period of pregnancy, without any warning signs. It can start at any time and the period before and after birth can cause the symptoms to become more severe. It is a pretty rare disease, according to clinical data. Only about one woman in 50000 develops pemphigoid gestationis during pregnancy.

Women of all races and ages can be affected but white women seem to be the most vulnerable. Women who already suffer from other autoimmune diseases are a lot more likely to develop it. The risk is also higher for those who have used oral contraceptives or have given birth several times.

Causes and risk factors

Pemphigoid is one of the poorly understood autoimmune diseases. It is triggered by a mistake of the immune system, which starts attacking the body tissues. Pemphigoid begins when this happens to the tissues located immediately under the skin. As a result, the skin layers separate and the visible effect is the appearance of blisters. Like all autoimmune diseases, pemphigoid remains a mystery and nobody is certain what causes the mistaken immune response.

Even if the exact mechanism is not known, some triggers of the disease can be identified. These are usually aggressive forms of treatment such as radiation therapy, ultraviolet light therapy or powerful medication. Elders have a much higher risk than adults or youngsters and people who already suffer from other forms of autoimmune disease are more likely to develop pemphigoid as well.

What are the symptoms of pemphigoid?

The only symptoms of pemphigoid are the rashes and blisters visible at skin level. The duration, intensity and location of the blisters are variable from one person to another and also depend on the condition's type. In most cases, it is a cyclical disease, with outbreaks followed by periods of remission.

Treatment options

There is no cure for pemphigoid itself but the symptoms can be treated quite effectively. In most cases, the first treatment is corticosteroids, either applied topically or taken orally. These compounds have strong anti-inflammatory effects, which calm down itching in the area and speed up the healing of blisters. It is usually a short-term treatment, stopped as soon as the blisters heal, because it can cause serious side effects if used for a long period of time.

Corticosteroids can be combined with drugs that reduce the reaction of the body's immune system. This prevents it from attacking healthy cells but it can be a very risky strategy, since it decreases resistance against other infections as well. Tetracycline or other antibiotics are sometimes prescribed to compensate for the suppressed immune system and fight infections.

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