Pemphigus

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. It's most common in people middle-aged or older.

Pemphigus is easier to control if caught and treated early. It's usually treated with medicines that you take long term. The sores may heal slowly or not at all. The condition can become life-threatening if the sores become infected.

Pemphigus causes blisters on the skin and mucous membranes. The blisters break easily, leaving open sores. The sores may become infected and ooze.

The symptoms of two common types of pemphigus are as follows:

• Pemphigus vulgaris. This type usually begins with blisters in the mouth and then on the skin or genital mucous membranes. They're often painful but don't itch. Blisters in the mouth or throat may make it hard to talk, drink and eat.
• Pemphigus foliaceus. This type causes blisters on the chest, back and shoulders. The blisters may be itchy or painful. Pemphigus foliaceus doesn't cause mouth blisters.

Pemphigus is distinct from bullous pemphigoid, which is another type of blistering skin condition that affects older adults.

When to see a doctor

See a healthcare professional if you have blisters that don't heal in the mouth or on the skin or genital mucous membranes.

Pemphigus is an autoimmune disorder, which means that your immune system mistakenly attacks healthy cells in your body. With pemphigus the immune system attacks cells in the skin and mucous membranes.

Pemphigus isn't passed from one person to another. In most cases, it's unknown what causes the disease to arise.

Rarely, the disease can develop as a side effect of medicines, like penicillamine and certain blood pressure drugs. This type of the condition usually clears up when the medicine is stopped.

The risk of pemphigus increases if you're middle-aged or older. The condition also is more common in people of Jewish, Indian, southeast European or Middle Eastern ancestry.

Possible complications of pemphigus include:

• Infection of the skin.
• Infection that spreads to your bloodstream, also called sepsis. This type of infection can be life-threatening.
• Scarring and changes in skin color after the affected skin heals. This is called postinflammatory hyperpigmentation when the skin darkens and postinflammatory hypopigmentation when the skin loses color. People with brown or Black skin have a higher risk of long-term skin color changes.
• Malnutrition, because painful mouth sores make it difficult to eat.
• Side effects from the medicine used to treat pemphigus. Examples are high blood pressure and infection.
• Death, rarely, if certain types of pemphigus are left untreated.

Your healthcare professional may start by talking with you about your medical history and symptoms and examining the affected area. In addition, you may have tests, including:

• A biopsy. A biopsy is a procedure to remove a sample of tissue for testing in a lab. To test for pemphigus, a piece of the blister is used.
• Blood tests. One purpose of these tests is to detect and identify antibodies in your blood that are known to be present with pemphigus.

Your healthcare professional may refer you to a specialist in skin conditions. This type of specialist is a dermatologist.

Treatment for pemphigus usually starts with medicines to ease symptoms and prevent new blisters. These may include steroids and medicines that target the immune system. If your symptoms were caused by use of certain medicines, stopping that medicine may be enough to clear up your symptoms.

Some people may need a hospital stay to receive fluids, nutrition or other treatments.

Medicines

Your healthcare professional may suggest one or more of the following medicines. The choice of medicines depends on the type of pemphigus you have, how severe your symptoms are and whether you have other medical conditions.

• Corticosteroids. For people with mild disease, corticosteroid cream or injections may be enough to control it. For others, the main treatment is a corticosteroid medicine taken by mouth, such as prednisone pills.

  Using corticosteroids for a long time or in high doses may cause severe side effects. These include diabetes, bone loss, an increased risk of infection, stomach ulcers and a shift of body fat. This shift in fat can lead to a round face, also called moon face. To avoid these side effects, steroids may be used only for short periods of time to control flare-ups. And other medicines that target the immune system may be used long term to control the disease.

• Medicines that target the immune system. Some medicines can stop your immune system from attacking healthy tissues. Examples are azathioprine (Imuran, Azasan), mycophenolate (Cellcept) and cyclophosphamide. These too can have severe side effects, including an increased risk of infection.
• Other medicines. If first line medicines aren't helping you, your healthcare professional may suggest another drug, such as dapsone, intravenous immunoglobulin or rituximab-pvvr (Ruxience). You may need antibiotics to treat infections.

Many people with pemphigus get better, especially if treatment is started early. But it may take years and can require taking medicine for a long time.

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

• Following wound care instructions. Taking good care of your wounds can help prevent infection and scarring. Talk with your healthcare professional about how best to care for your wounds and control pain.
• Washing your skin gently. Use mild soap, rinse and apply lotion afterward.
• Protecting your skin. Avoid activities that may hurt the skin. And protect it from too much heat and sun, even on cool, cloudy or hazy days.
• Avoiding certain foods. Blisters in the mouth could be made worse by spicy, hot or crunchy foods.
• Taking care of your teeth and gums. Regular follow-up with a dentist is important for people with pemphigus. Talk with your dentist about how to best take care of your teeth and gums.

Pemphigus may be difficult to live with, especially if it affects your daily activities or causes lost sleep or stress. You may find it helpful to talk to others with the disease. You may want to connect with a support group in person or online. Ask your healthcare professional for suggestions.

You're likely to first see your regular healthcare professional. You may then be referred to a healthcare professional who specializes in skin disorders. This type of doctor is called a dermatologist.

Here's some information to help you get ready for your appointment.

What you can do

Before your appointment, make a list of:

• Symptoms you've been having and for how long.
• Key personal information, including any major stresses or recent life changes.
• All medicines, vitamins and supplements you take, including doses.
• Questions to ask your healthcare professional.

For pemphigus, some basic questions to ask your healthcare professional include:

• What's the most likely cause of my symptoms?
• Are there other possible causes?
• Do I need any tests? Do these tests require any special preparation?
• What treatments are available, and which do you recommend?
• What side effects can I expect from treatment?
• How long will it take for the blisters to heal? Will they leave scars?
• Will the blisters come back again?
• What can I do for the pain?
• I have other health conditions. How can I best manage them together?
• Is there a generic alternative to the medicine you're prescribing me?
• Do you have any brochures or other printed material I can take with me? What websites do you recommend?

What to expect from your doctor

Your healthcare professional is likely to ask you a number of questions, such as:

• When did you first begin experiencing symptoms?
• Does anything seem to improve your symptoms?
• What steps have you taken to treat this condition yourself?
• Have any of these measures helped?
• Have you ever been treated by a healthcare professional for this condition?
• If so, did you use any prescription treatments for this skin condition? If so, do you remember the name of the medicine and the dosage you were prescribed?
• Did you have a skin biopsy?