Definition: is a group of rare skin disorders that cause blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals.
The two main types are: vulgar (pemphigus vulgaris) and foliaceous (pemphigus foliaceus) . Pemphigus vulgaris usually begins in the mouth. It can be painful.
Pemphigus foliaceus affects the skin and tends to cause more itching than painful. Pemphigus can occur at any age, but is more common in middle-aged or older people.
Pemphigus should not be confused with bullous pemphigoid , another condition of the skin with blisters.
Usually, a chronic condition, pemphigus is better controlled by early diagnosis and treatment. The treatment may include medications and therapies similar to those used for severe burns.
Pemphigus vulgaris is an autoimmune, intraepithelial, blistering disease that affects the skin and mucous membranes.
It is mediated by circulating autoantibodies directed against surfaces of keratinocyte cells. A life-threatening disease, it has a mortality rate of approximately 5-15%.
Pemphigus is characterized by blisters on the skin and mucous membranes. The blisters break easily, leaving open sores that can ooze and become infected.
The signs and symptoms of the two main types of pemphigus are the following:
Pemphigus vulgaris . This type usually begins with blisters in the mouth and then on the skin or genital mucous membranes.
The blisters are typically painful, but they do not itch. Blisters in the mouth or throat can make it difficult to swallow and eat.
Pemphigus foliáceo . This type does not usually affect the mucous membranes. And the blisters tend not to be painful. This condition can affect any part of the body, but most blisters are on the chest, back and shoulders.
The blisters make the skin crunchy and itchy.
When to see a doctor
Consult your doctor if you develop blisters inside your mouth or on your skin. If you have already been diagnosed with pemphigus and are receiving treatment, consult your doctor if it develops:
- New blisters or sores
- A rapid spread of the number of sores.
- Fever, redness or swelling, which may indicate infection.
- Weakness or pain in the muscles or joints.
Pemphigus is an autoimmune disorder . It is not contagious. In most cases, it is not known what triggers the disease.
Normally, your immune system attacks foreign invaders, such as harmful viruses and bacteria. But in pemphigus, your immune system mistakenly produces antibodies that attack healthy cells in the skin and mucous membranes.
Rarely, pemphigus develops as a side effect of medications, such as certain medications for blood pressure. This type of pemphigus usually goes away when the medication is stopped.
Your risk of pemphigus increases if you are middle-aged or older. People of Jewish descent have an increasing incidence of pemphigus vulgaris.
The open sores of pemphigus make it very vulnerable to infection, which, if it spreads to your bloodstream, can be fatal. Possible complications of pemphigus include:
- Skin infection
- Infection that spreads to your bloodstream (sepsis).
- Gum disease and tooth loss, if you have blisters in your mouth.
- Side effects of medication, such as high blood pressure and infection.
- Death by infection.
Blisters occur with a number of conditions, so pemphigus can be difficult to diagnose. Your doctor will ask for a complete medical history and examine your skin and mouth.
In addition, he or she can:
Check if there is peeling of the skin . Your doctor will lightly rub a patch of normal skin near the area with blisters with a cotton swab or finger.
If you have pemphigus, it is likely that the upper layers of your skin fall off.
Make a skin biopsy . In this test, a piece of tissue is removed from a blister and examined under a microscope.
Blood tests . One of the objectives of these tests is to detect and identify antibodies in your blood known as desmogleins.
These antibodies are usually elevated when pemphigus is first diagnosed. The levels of these antibodies usually decrease as symptoms improve.
Ask for an endoscopy exam . If you have pemphigus vulgaris, your doctor may have you undergo an endoscopy to check for sores in the throat. This procedure involves inserting a flexible tube (endoscope) into the throat.
Treatment usually begins with medications that are meant to reduce signs and symptoms and prevent complications.
It is usually more effective when it starts as soon as possible. The treatment may also involve a stay in the hospital. Pemphigus can be life-threatening.
The following prescription medications can be used alone or in combination, depending on the type and severity of your pemphigus:
REMEMBER NEVER AUTOMATED – ALWAYS CONSULT YOUR DOCTOR
Corticosteroids . For people with mild illness, corticosteroid cream may be enough to control it. For others, the mainstay of treatment is corticosteroids, such as prednisone pills.
The use of corticosteroids for a long time or in high doses can cause serious side effects, such as increased blood sugar, bone loss, an increased risk of infection, cataracts, glaucoma and a redistribution of body fat.
Immunosuppressants . Medications such as azathioprine (Imuran) or mycophenolate mofetil (CellCept) help keep your immune system attacking healthy tissue. They can have serious side effects, including an increased risk of infection.
Biological therapies . Your doctor may suggest a medicine called rituximab (Rituxan) if other medications do not help or are difficult to tolerate.
This medicine is given as an injection. It targets the white blood cells responsible for the production of pemphigus antibodies.
Antibiotics, antiviral and antifungal. These can be used to control or prevent infections due to pemphigus.
Other medications . Other drugs that alter the immune system can be effective. These include dapsone and intravenous immunoglobulin.