Pemphigoid

Description:
Pemphigoid is a rare blistering disorder, which usually occurs in later life, the average age of onset being over 70 years.

The blisters come up on the skin and, less often, in the mouth too. This is in contrast to a related condition known as ‘mucous membrane pemphigoid’ in which the brunt of the trouble is borne by the moist surfaces of the body (the mucous membranes) such as the eyes, inside the nose and mouth, and the genitals. Yet another type of pemphigoid (pemphigoid gestationis) occurs during pregnancy. This leaflet will not discuss mucous membrane pemphigoid or pemphigoid gestationis further.

It is a group of rare autoimmune blistering skin diseases. As its name indicates, pemphigoid is similar in general appearance to pemphigus,  But, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.

Pemphigoid is more common than pemphigus, and is slightly more common in women than in men. It is also more common in people over 60 years of age than it is in younger people.

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Types of pemphigoid:

All types of pemphigoid are caused by your immune system attacking healthy tissue. They appear as rashes and fluid-filled blisters. The types of pemphigoid differ in terms of where on the body the blistering occurs and when it occurs.

Bullous pemphigoid:

In cases of bullous pemphigoid — the most common of the three types — the skin blistering happens most commonly on the arms and legs where movement occurs. This includes the areas around the joints and on the lower abdomen.

Cicatricial pemphigoid:

Cicatricial pemphigoid, also called mucous membrane pemphigoid, refers to blisters that form on the mucous membranes. This includes the:

* mouth
* eyes
* nose
* throat
* genitals

The most common sites affected are the mouth and eyes. The rash and blistering may begin in one of these areas and spread to the others if left untreated. If it’s left untreated in the eyes, it may cause scarring, which in turn may lead to blindness.

Pemphigoid gestationis:

When blistering occurs during or shortly after pregnancy, it’s called pemphigoid gestationis. It was formerly called herpes gestationis, although it’s not related to the herpes virus.

The blistering typically develops during the second or third trimester, but may occur at any time during pregnancy, or up to six weeks after delivery. Blisters tend to form on the arms, legs, and abdomen.
a quarter of patients with pemphigoid have blisters or raw areas in the mouth.

Symptoms:
The most common symptom of pemphigoid is blistering that occurs on the arms, legs, abdomen, and mucous membranes. Hives and itching are also common. The blisters have certain characteristics, regardless of where on the body they form:

* a red rash develops before the blisters
* the blisters are large and filled with fluid that’s usually clear, but may contain some blood
* the blisters are thick and don’t rupture easily
* the skin around the blisters may appear normal, or slightly red or dark
* ruptured blisters are usually sensitive and painful

Itching is common. The raw areas left when the blisters break can be sore, both on the skin and in the mouth.

Causes:
Pemphigoid is an autoimmune disease. This means that your immune system mistakenly begins to attack your healthy tissues. In the case of pemphigoid, your immune system creates antibodies to attack the tissue just below your outer layer of skin. This causes the layers of skin to separate and results in painful blistering. It’s not fully understood why the immune system reacts this way in people living with pemphigoid.

In many cases, there’s no specific trigger for pemphigoid, either. In some instances, however, it may be caused by:

* certain medications
* radiation therapy
* ultraviolet light therapy

People with other autoimmune disorders are found to be at a higher risk for developing pemphigoid. It’s also more common in the elderly than in any other age group, and seems to occur slightly more in women than men.

Diagnosis:
Itching is common. The raw areas left when the blisters break can be sore, both on the skin and in the mouth.

What does pemphigoid look like?

  • A rash may be present for some weeks before any blisters come up. At that stage, the rash may look like an odd eczema or, more commonly, like the red weals of nettle rash (urticaria).*  When blisters do come up, they often do appear on red patches. Any part of the skin can be involved, but the most common sites for the blisters are the body folds and the skin on the abdomen. In severe cases, the blisters can occur all over the body.
  • The blisters have thick roofs, and can get quite large and tense before they burst. Most contain clear fluid but in a few this is bloodstained. When the blisters heal up, they do so without leaving scars.

* Roughly a quarter of patients with pemphigoid have blisters or raw areas in the mouth.

Usually the look of the rash is enough to make the diagnosis, but it is essential to get further proof.

* Examination of a biopsy taken from a small and early blister will show that it has come up just under the outermost layer of the skin (i.e. it is a ‘subepidermal’ blister).

* Special testing (immunofluorescence) of a sample of normal skin (i.e. taken from an area where there is no blister) will show up a layer of antibodies that also lies just under the outer layer of the skin.

* The same pemphigoid antibodies can be detected circulating in the blood.

Treatment:
No. Treatment helps a lot, but controls the condition rather than curing it completely. However, pemphigoid does often go away by itself after one to five years.

Corticosteroids, either in pill or topical form, will likely be the first treatment the doctor prescribes. These medications reduce inflammation and can help to heal the blisters and relieve itching. However, they can also cause significant side effects, especially from long-term use, so your doctor will taper you off of the corticosteroids after the blistering clears up.

Another treatment option is to take medication that suppresses the patient’s immune system, often in conjunction with the corticosteroids. Immunosuppressants help, but they may put him or her at risk for other infections. Certain antibiotics, such as tetracycline, may also be prescribed to reduce inflammation and infection.

Risk Factors:
People with other autoimmune disorders are found to be at a higher risk for developing pemphigoid. It’s also more common in the elderly than in any other age group, and seems to occur slightly more in women than men.

Disclaimer : The information presented herein is intended for educational purposes only. Individual results may vary, and before using any supplement, it is always advisable to consult with your own health care provider.

Resources:
https://en.wikipedia.org/wiki/Pemphigoid
http://www.britishskinfoundation.org.uk/Skinformation/PEMPHIGOID.aspx
https://www.healthline.com/health/bullous-pemphigoid

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