Overview

Pemphigus 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 pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris usually starts in your mouth. It can be painful. Pemphigus foliaceus affects the skin and tends to be more itchy than painful. Pemphigus can occur at any age, but it's most often seen in people who are middle-aged or older.

Pemphigus is not to be confused with bullous pemphigoid, another blistering skin condition. Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment. Treatment may include medications and therapies similar to those used for severe burns.

Source: http://www.mayoclinic.com

Symptoms

Pemphigus is characterized by blisters on your skin and mucous membranes. The blisters rupture easily, leaving open sores, which may ooze and become infected.

The signs and symptoms of the two main types of pemphigus are as follows:

• Pemphigus vulgaris. This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. The blisters typically are painful, but don't itch. Blisters in your mouth or throat may make it hard to swallow and eat.
• Pemphigus foliaceus. This type doesn't usually affect mucous membranes. And the blisters tend not to be painful. This condition may affect any skin, but most blisters are on the chest, back and shoulders. The blisters cause the skin to be crusty and itchy.

When to see a doctor

See your doctor if you develop blisters inside your mouth or on your skin. If you've already been diagnosed with pemphigus and are receiving treatment, see your doctor if you develop:

• New blisters or sores
• A rapid spread in the number of sores
• Fever, redness or swelling, which may indicate infection
• Chills
• Weakness or achy muscles or joints

Source: http://www.mayoclinic.com

Causes

Pemphigus is an autoimmune disorder. It's not contagious. In most cases, it's unknown 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 your skin and mucous membranes.

Rarely, pemphigus develops as a side effect of medications, such as certain blood pressure drugs. This type of pemphigus usually disappears when the medicine is stopped.

Source: http://www.mayoclinic.com

Diagnosis

Blisters occur with a number of conditions, so pemphigus can be difficult to diagnose. Your doctor will ask you for a complete medical history and examine your skin and mouth. In addition, he or she may:

• Check for skin peeling. Your doctor will lightly rub a patch of normal skin near the blistered area with a cotton swab or finger. If you have pemphigus, the top layers of your skin are likely to shear off.
• Do a skin biopsy. In this test, a piece of tissue from a blister is removed and examined under a microscope.
• Run blood tests. One purpose of these tests is to detect and identify antibodies in your blood known as desmogleins. These antibodies are often elevated when pemphigus is first diagnosed. The levels of these antibodies usually goes down as symptoms improve.
• Order an endoscopy exam. If you have pemphigus vulgaris, your doctor may have you undergo endoscopy to check for sores in the throat. This procedure involves inserting a flexible tube (endoscope) down your throat.

Source: http://www.mayoclinic.com

Complications

The open sores of pemphigus make you highly vulnerable to infection, which, if it spreads to your bloodstream, can be fatal. Possible complications of pemphigus include:

• Infection of your skin
• Infection that spreads to your bloodstream (sepsis)
• Gum disease and tooth loss, if you have blisters in your mouth
• Medication side effects, such as high blood pressure and infection
• Death from infection

Lifestyle and home remedies

Here are steps you can take to improve your skin and overall health:

• Follow your doctor's wound care instructions. Taking good care of your wounds can help prevent infection and scarring.
• Use talcum powder. Generously sprinkling talcum powder on your sheets may help keep oozing skin from sticking.
• Take your medicines as prescribed. Stopping or changing the dose could cause your condition to worsen.
• Clean towels, linens and clothing. Frequent cleaning of these items helps prevent your blisters and sores from becoming infected. Also, don't share such items with others.
• Protect your skin. Avoid activities that may hurt or contaminate your wounds, such as playing contact sports or using a hot tub.
• Avoid certain foods. Blisters in your mouth could be triggered or irritated by some foods, such as garlic, onion and abrasive foods.
• Minimize sun exposure. Ultraviolet light may trigger new blisters.
• Talk with your dentist about maintaining good oral health. If you have blisters in your mouth, it may be difficult to brush your teeth properly. Ask your dentist what you can do to protect your oral health.
• Ask your doctor if you need calcium and vitamin D supplements. Corticosteroids can affect your calcium and vitamin D needs, so ask your doctor if you need a calcium supplement or other additional nutrients.

Source: http://www.mayoclinic.com

Coping and support

Pemphigus may be difficult to live with, especially if it affects your daily activities or makes you feel embarrassed. You may find it helpful to talk to others with the disease. You can find in-person or virtual support groups. Ask your doctor for suggestions.

Source: http://www.mayoclinic.com

Risk factors

Your risk of pemphigus increases if you're middle-aged or older. People of Jewish ancestry have an increased incidence of pemphigus vulgaris.

Source: http://www.mayoclinic.com