Description

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 is more common in people of middle age or older.

Pemphigus is easier to control if it is detected and treated in time. It is usually treated with medicines that you take in the long term. The blisters heal slowly or not at all. The disease can be deadly if infected ulcers.

Symptoms

Pemphigus cause blisters on the skin and mucous membranes. The blisters break easily, leaving open wounds. The ulcers can become infected and ooze.

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

  • Pemphigus vulgaris. This type usually begins with blisters in the mouth and then in the skin or mucous genitals. They are often painful, but aren't biting. Blisters in the mouth or throat can make it difficult to talk, drink and eat.
  • Pemphigus foliaceus. This type of cause blisters on the chest, back and shoulders. The sores may be itchy or painful. Pemphigus foliaceus does not cause blisters in the mouth.

Pemphigus is distinct from pemphigus, which is another type of blisters on the skin condition that affects older adults.

When to see a doctor

Consult a health care professional if you have blisters that do not heal in the mouth or on the skin or genital mucous membranes.

Causes

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

Pemphigus is not transmitted from one person to another. In most cases, it is unknown what causes the disease to arise.

In rare cases, the disease can develop as a side effect of medications, including penicillamine, and certain blood pressure drugs. This type of condition usually goes away when the medicine is stopped.

Risk factors

The risk of pemphigus increases if you are middle-aged or older. The condition is more common in the people of the Jews, India, southeast European or Middle Eastern ancestry.

Complications

The possible complications of pemphigus include:

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

Diagnosis

Your health care professional may start by talking with you about your symptoms and medical history and examination of 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. For the test of pemphigus, a piece of the ampoule is used.
  • Blood tests. One of the purposes of these tests is to detect and identify antibodies in your blood that are known to present with bullous pemphigoid.

Your healthcare provider may refer you to a specialist in diseases of the skin. This type of specialist is a dermatologist.

Treatment

Treatment for pemphigus usually begins with medications to alleviate symptoms and prevent blisters. These may include steroids and drugs that target the immune system. If your symptoms were caused by the use of certain drugs, to stop the medicine, it may be sufficient to clarify their symptoms.

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

Medications

Your health care professional may suggest one or more of the following medications. The choice of drugs depends on the type of pemphigus you have, the severity of your symptoms and whether you have other medical conditions.

  • Corticosteroids.For people with mild disease, corticosteroid cream or injections may be sufficient for control. For others, the main treatment is a corticosteroid medication that is taken by mouth (such as prednisone tablets. The use of corticosteroids for a long period of time or at high doses can cause serious side effects. These include diabetes, bone loss, an increased risk of infection, stomach ulcers, and a change of body fat. This change 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 for the control of outbreaks. And other drugs that target the immune system can be used long-term control of the disease.
  • The drugs that target the immune system. Some medicines may stop the immune system attacks the healthy tissues. Examples include azathioprine (Imuran, Azasan), mycophenolate (Cellcept) and cyclophosphamide. These can also have serious side effects, including an increased risk of infection.
  • Other medications. If the first line of drugs are not helping you, your health care professional may suggest another medication, such as dapsone, intravenous immunoglobulin or rituximab-pvvr (Ruxience). You may need antibiotics to treat infections.

Corticosteroids. For people with mild disease, corticosteroid cream or injections may be sufficient for control. For others, the main treatment is a corticosteroid medication that is taken by mouth (such as prednisone tablets.

The use of corticosteroids for a long period of time or at high doses can cause serious side effects. These include diabetes, bone loss, an increased risk of infection, stomach ulcers, and a change of body fat. This change 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 for the control of outbreaks. And other drugs that target the immune system can be used long-term control of the disease.

Many people with pemphigus improve, especially if treatment is started early. But it can take years and may need to take medicine for a long time.

Lifestyle and home remedies

Here are the steps that you can take to improve your skin and health in general:

  • After the wound care instructions. Taking good care of your injuries can help prevent infection and scarring. Talk with your health care professional about how best to care for your wounds and pain control.
  • Wash the skin gently. Use a mild soap, rinse and apply the lotion after.
  • The protection of your skin. Avoid activities that may damage the skin. And to protect it from too much heat and the sun, even on cool, cloudy or foggy days.
  • Avoid certain foods. Blisters in the mouth could be aggravated by the spicy, hot or crunchy foods. The care of the teeth and gums.A Regular follow-up with a dentist is important for people with pemphigus. Talk with your dentist about how to take care of your teeth and gums.
  • The care of the teeth and gums. A Regular follow-up with a dentist is important for people with pemphigus. Talk with your dentist about how to take care of your teeth and gums.

Coping and support

Pemphigus can be difficult to live with, especially if it affects your daily activities or causes of loss of sleep or stress. You may find it helpful to talk with other people with the disease. It is possible that you want to connect with a support group in person or online. Ask your health care professional for suggestions.

Preparing for your appointment

It is likely that you first consult your regular healthcare provider. Then, you may be referred to a health professional who specializes in disorders of the skin. This type of doctor is called a dermatologist.

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

What you can do

Before your appointment, make a list of:

  • The symptoms that you've had and for how long.
  • Key personal information, including any major stresses or recent life changes.
  • All the drugs, vitamins, and supplements you are taking, including dosage.
  • Questions to ask your health care professional.

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

  • What is the most likely cause of my symptoms?
  • There are 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? They are going to leave scars?
  • Are the blisters can come back again?
  • What can I do to relieve 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 that I can take with me? What sites do you recommend?

What to expect from your doctor

Your healthcare provider is likely to ask a series of questions, such as:

  • When did you first begin experiencing symptoms?
  • Nothing seems to improve the symptoms?
  • What measures have been adopted for the treatment of this condition yourself?
  • Have any of these measures helped?
  • Have you ever been treated by a health care practitioner for this condition?
  • If so, does the use of any other prescription treatments for this skin condition? If so, do you remember the name of the medication and dosage prescribed to you?
  • Do you have a skin biopsy?
Symptoms and treatment of Pemphigus