Overview

Overview of Pemphigus

Pemphigus is a disease that causes blistering of the skin and the inside of the mouth, nose, throat, eyes, and genitals. The disease is rare in the United States.

Pemphigus is an autoimmune disease in which the immune system mistakenly attacks cells in the top layer of the skin (epidermis) and the mucous membranes. People with the disease produce antibodies against desmogleins, proteins that bind skin cells to one another. When these bonds are disrupted, skin becomes fragile, and fluid can collect between its layers, forming blisters.

There are several types of pemphigus, but the two main ones are:

  • Pemphigus vulgaris, which normally affects the skin and mucous membranes such as the inside of the mouth.
  • Pemphigus foliaceus, which only affects the skin.

There is no cure for pemphigus, but in many cases, it is controllable with medications.

Who Gets

Who Gets Pemphigus?

You are more likely to get pemphigus if you have certain risk factors. These include:

  • Ethnic background. While pemphigus occurs across ethnic and racial groups, some populations are at greater risk for certain types of the disease. People of Jewish (especially Ashkenazi), Indian, Southeast European, or Middle Eastern descent are more susceptible to pemphigus vulgaris.
  • Geographic location. Pemphigus vulgaris is the most common type worldwide, but pemphigus foliaceus is more common in some places, such as certain rural regions of Brazil and Tunisia.
  • Sex and age. Women get pemphigus vulgaris more frequently than men do, and the age of onset is usually between 50 and 60 years old. Pemphigus foliaceus generally affects men and women equally, but in some populations, women get the disease more frequently than men do. While the age of onset of pemphigus foliaceus is usually between 40 and 60 years old, in some areas, symptoms may begin in childhood.
  • Genes. Scientists believe that the higher frequency of the disease in certain populations is partly due to genetics. For example, evidence shows that certain variants in a family of immune system genes called HLA are linked to a higher risk of pemphigus vulgaris and pemphigus foliaceus.
  • Medications. In rare cases, pemphigus has resulted from taking certain medicines, such as certain antibiotics and blood pressure medications. Medicines that contain a chemical group called a thiol have also been linked to pemphigus.
  • Cancer. Rarely, the development of a tumor—in particular a growth in a lymph node, tonsil, or thymus gland—can trigger the disease.

Types

Types of Pemphigus

There are two major forms of pemphigus, and they are categorized based on the layer of skin where the blisters form and where the blisters are found on the body. The type of antibody that attacks the skin cells also helps define the type of pemphigus.

The two main forms of pemphigus are:

  • Pemphigus vulgaris is the most common type in the United States. Blisters form in the mouth and other mucosal surfaces, as well as on the skin. They develop within a deep layer of the epidermis and are often painful. There is a subtype of the disease called pemphigus vegetans in which blisters form mainly in the groin and under the arms.
  • Pemphigus foliaceus is less common and only affects the skin. The blisters form in upper layers of the epidermis and may be itchy or painful.

Other rare forms of pemphigus include:

  • Paraneoplastic pemphigus. This type is characterized by sores in the mouth and on the lips, but blisters or inflamed lesions usually also develop on the skin and other mucosal surfaces. Severe lung problems may occur with this type. People with this type of the disease usually have a tumor, and the disease may improve if the tumor is surgically removed.
  • IgA pemphigus. A type of antibody called IgA causes this form. Blisters or pimple-like bumps often appear in groups or rings on the skin.
  • Drug-induced pemphigus. Certain medicines, such as some antibiotics and blood pressure medications, as well as drugs that contain a chemical group called a thiol, may bring on pemphigus-like blisters or sores. The blisters and sores usually go away when you stop taking the medication.

Pemphigoid is a disease that is different from pemphigus but shares some of its features. Pemphigoid produces a split where the epidermis and the underlying dermis meet, causing deep, rigid blisters that do not break easily.

Symptoms

Symptoms of Pemphigus

The main symptom of pemphigus is blistering of the skin and in some cases, the mucosal surfaces, such as the inside of the mouth, nose, throat, eyes, and genitals. The blisters are fragile and tend to burst, causing crusty sores. Blisters on skin may join together, forming raw-looking areas that are prone to infection and that ooze large amounts of fluid. The symptoms vary somewhat depending on the type of pemphigus.

  • Pemphigus vulgaris blisters often start in the mouth, but later on, they can develop on the skin. The skin may become so fragile that it peels off by rubbing a finger on it. Mucosal surfaces such as those of the nose, throat, eyes, and genitals may also be affected.
    Blisters form within the deep layer of the epidermis, and they are often painful.
  • Pemphigus foliaceus only affects the skin. Blisters often appear first on the face, scalp, chest, or upper back, but they may eventually spread to other areas of skin on the body. The affected areas of skin may become inflamed and peel off in layers or scales. The blisters form in the upper layers of the epidermis, and they may be itchy or painful.

Causes

Causes of Pemphigus

Pemphigus is an autoimmune disorder that happens when the immune system attacks healthy skin. Immune molecules called antibodies target proteins called desmogleins, which help link neighboring skin cells to one another. When these connections are broken, skin becomes fragile and fluid can collect between layers of cells, forming blisters.

Normally, the immune system protects the body from infection and disease. Researchers do not know what causes the immune system to turn on the body’s own proteins, but they believe that both genetic and environmental factors are involved. Something in the environment may trigger pemphigus in people who are at risk because of their genetic makeup. In rare cases, pemphigus may be caused by a tumor or by certain medications.

Diagnosis

Diagnosis of Pemphigus

Early diagnosis is important, so if you have blisters on the skin or in the mouth that do not go away, it is important to see a doctor as soon as you can. Your doctor may try to rule out other conditions first, since pemphigus is a rare disease. Your doctor may:

  • Take your medical history, and give you a physical exam. A dermatologist (a doctor who specializes in conditions of the skin, hair, and nails) may ask you about your medical history and look at the appearance and location of blisters. He or she may run a finger or cotton swab over the surface of your skin to see if it shears off easily.
  • Take a tissue sample. Your doctor may take a sample from one of your blisters to:
    • Examine it under the microscope to look for cell separation and to determine the layer of skin in which the cells are separated.
    • Determine which antibodies attacked the skin.
  • Take a blood sample. Antibody levels in your blood can help determine the severity of the disease. This blood test may also be used later on to see if treatment is working.

Treatment

Treatment of Pemphigus

There is no cure for pemphigus, but treatment can control the disease in most people. The initial goal of treatment is to clear existing blisters and help prevent relapses. Treatment typically depends on the severity and stage of the disease.

Symptoms of pemphigus may go away after many years of treatment, but most people need to continue taking medications to keep the disease under control. Treatment for pemphigus may involve the following medications:

  • Corticosteroids. These anti-inflammatory medicines are a mainstay of treatment for pemphigus. They may be applied topically as a cream or ointment, or by mouth or injection (systemically). Most people will be prescribed systemic corticosteroids, at least initially, to bring the disease under control. Because they are potent drugs, your doctor will prescribe the lowest dose possible to achieve the desired benefit. 
  • Immunosuppressants. These help suppress or curb the overactive immune system.
  • Biologic response modifiers. These target specific immune messages and interrupt the signal, helping to stop the immune system from attacking the skin.
  • Antibiotics, antivirals, and antifungal medications to control or prevent infections.

If the above treatments do not work or pemphigus is severe, other treatments may be considered. These treatments include:

  • Plasmapheresis or immunoadsorption, which remove damaging antibodies from the blood.
  • Intravenous immunoglobulin therapy, in which you are given pooled antibodies from 1,000 or more healthy blood donors.

Be sure to report any problems or side effects from medications to your doctor.

In some cases, a person with pemphigus may need to be hospitalized to treat health problems that the disease or its treatment can cause. Widespread sores on the skin can result in dehydration or infection, and painful blisters in the mouth can make it difficult to eat. In the hospital, you may be given an IV to replace lost fluids, to get much-needed nutrition, and to treat infection.

Who Treats

Who Treats Pemphigus?

The following health care providers may diagnose and treat pemphigus:

  • Dermatologists, who specialize in conditions of the skin, hair, and nails.
  • Dentists, who can tell you how to take care of your gums and teeth if you have blisters in your mouth.
  • Mental health professionals, who help people cope with difficulties in the home and workplace that may result from their medical conditions. 
  • Ophthalmologists, in cases where the eyes are affected. Ophthalmologists specialize in treating disorders and diseases of the eye. 
  • Primary care doctors, such as a family physician or internal medicine specialist, who coordinate care between the different health care providers and treat other problems as they arise.

Living With

Living With Pemphigus

Blisters in the mouth may make brushing and flossing your teeth painful, so talk to your dentist about ways to keep your teeth and gums healthy. Avoid foods that irritate your mouth blisters. Your dermatologist may recommend baths and wound dressings to help heal the sores and blisters.

Pemphigus and its treatments can be debilitating and cause lost time at work, weight loss, sleep problems, and emotional distress. A mental health professional or a support group may help you cope with the disease.

Remember to follow the recommendations of your health care providers.

Research Progress

Research Progress Related to Pemphigus

The NIAMS Dermatology Branch conducts clinical and basic research of the skin and skin diseases, such as:  

  • The skin as an organ that has immunological properties.  
  • Inflammatory skin diseases. 
  • The human microbiome and its influence in healthy people and those with skin diseases. 
  • Skin stem cells. 

In addition, the NIAMS conducts and funds research on pemphigus, including:

  • Genetics: Evidence indicates that genetics partly account for the higher frequency of pemphigus in certain populations, but the role of genes remains unclear. It appears that multiple genes are involved in the disease, and research is underway to identify them.
  • Environmental factors: Scientists believe that environmental factors may trigger pemphigus in people who are genetically at risk. They are working to assess the impact on the disease of various factors, such as exposure to dust, chemicals, or the bite of certain insects.
  • Immune system targets: Pemphigus is caused by an aberrant immune attack on skin. A number of investigators are pursuing strategies to bring the disease under control by targeting immune cells.
    • B cells: Several efforts are aimed at characterizing B cells, immune cells that play a key role in the autoimmune attack on skin in people with the disease. By providing information on how these cells turn on the body and attack healthy skin, this work may lead to new treatment strategies. In addition, clinical trials are underway to test the efficacy of certain B cell cancer medicines on pemphigus.
    • T cells: When the body is infected with a pathogen, a population of immune cells called regulatory T cells helps to shut down the immune response once the threat is gone. Researchers are trying to find ways to engineer these cells to quell the autoimmune attack in pemphigus.

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