What is pemphigoid?
Pemphigoid is a family of rare autoimmune diseases that causes blisters and rashes on the skin and mucous membranes. The body mistakenly sends antibodies to bind to skin cells. These antibodies set off a chain reaction that separates the lower layer of cells from the upper layers.
The condition can affect people of any age, but it tends to affect older adults. It can also develop during pregnancy or from the use of certain types of medications and therapies. While there is currently no cure for this disease, there are several treatment options.
All types of pemphigoid are caused by the immune system of aggressive healthy tissue. They appear as rashes and fluid-filled blisters. The types of pemphigoid differ in terms of where on the body the blisters occur and when they occur.
In bullous pemphigoid, the most common of the three types, blistering of the skin most often occurs on the arms and legs, where movement occurs. This includes the areas around the joints and in the lower abdomen.
Scar pemphigoid, also called mucosal pemphigoid, refers to blisters that form on the mucous membranes. This includes:
The most commonly affected sites are the mouth and eyes. The rash and blisters can start in one of these areas and spread to the others if left untreated. If left untreated in the eyes, it can cause scarring, which in turn can lead to blindness.
When blisters form during or shortly after pregnancy, it is called gestational pemphigoid. It used to be called herpes gestationis, although it is not related to the herpes virus.
Blisters usually develop during the second or third trimester but can occur at any time during pregnancy or up to six weeks after delivery. Blisters incline to form on the arms, legs, and abdomen.
Pemphigoids are not genetic (inherited), though there may be a genetic predisposition to developing the disease. A person who is hereditarily predisposed to a disorder carries a gene for the disease, but it may not be spoken unless it is triggered or “activated” in certain circumstances, for example, due to particular environmental factors. Currently, it is not possible to predict who can get these diseases.
It can present with itching, hive-shaped welts, and multiple blisters, called blisters. These are most often seen in:
The blisters can break open and become an open sore or ulcer. The fluid inside may be clear or contain some blood. The skin around the blisters may look normal or red. The blisters are usually located along the folds of the skin. You should seek medical courtesy from your healthcare provider if you think you might have bullous pemphigoid.
If there are characteristic blisters, doctors often diagnose bullous pemphigoid using a skin biopsy. For more unusual cases, such as those causing itchy rash without blisters, blood tests may also be required.
A doctor may order a biopsy to diagnose bullous pemphigoid and gestational pemphigoid. Bullous pemphigoid can be distinguished from other skin conditions that cause blisters by the presence of certain factors, such as:
- Head and neck are not affected
- Few or no mucous membrane symptoms
- Little or no bites or scars
The diagnosis of CP is usually made by a combination of a patient’s history, physical examination, and a biopsy of affected blisters or mucous tissue. Gestational pemphigoid is usually diagnosed by a skin biopsy. Doctors can differentiate the condition from other disorders by checking for the presence of antibodies in skin and blood samples.
Thyroid tests are often done to distinguish gestational pemphigoid from other autoimmune disorders that can cause similar symptoms, such as Grave’s disease.
It cannot be cured, but treatments are often very successful in relieving symptoms. Corticosteroids, also in pill or topical form, will likely be the first treatment your physician prescribes. These medications reduce inflammation and can help heal blisters and relieve itching. However, they can also cause significant side effects, especially from long-term use, so your doctor will lower your corticosteroid dose after the blisters disappear.
Another treatment option is to take medications that suppress your immune system, often in conjunction with corticosteroids. Immunosuppressants help but can put you at risk for other infections. Sure antibiotics, such as tetracycline, may also be prescribed to reduce inflammation and infection.
Your risk of this disease increases if you are middle-aged or older. The condition tends to be more common in people of Jewish or Middle Eastern descent.
Possible complications may include:
- Infection of your skin
- Infection that spreads to the bloodstream (sepsis)
- Malnutrition, because painful sores in the mouth make it difficult to eat
- Side effects of medications, such as high blood pressure and infections
- Death, if certain types of pemphigus are not treated