Definition

Pemphigus is a group of disorders that affect the skin. The attacks cause blisters and burn-like wounds on skin and mucous membranes like the mouth. There are 3 major forms of the disease:

• Pemphigus vulgaris—most common type of pemphigus
• Pemphigus foliaceus
• Paraneoplastic pemphigus—most serious type, usually occurs with cancer

Causes

Pemphigus is an autoimmune disorder. The immune system attacks healthy skin and mucus membranes. The attack causes the sores on the skin.

It is not clear what causes the immune system to attack normal body tissue. It is likely due to a combination of genetic and environmental factors. For some, medication may be the cause.

Risk Factors

Pemphigus is more common in people of Jewish or Mediterranean descent. Other factors that may increase your chance of pemphigus include:

• Family members with pemphigus
• A history of having autoimmune diseases, such as myasthenia gravis, systemic lupus erythematosus, or thymoma
• Regular use of certain medications:
  • Chelating agents, such as penicillamine
  • ACE inhibitors
  • Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin
  • Antibiotics, such as penicillin
  • Antiseizures, such as phenobarbital
  • Thiols

Symptoms

Pemphigus may occur over a small or large section of the skin. Itching and pain are common symptoms. Other symptoms will vary according to the type of pemphigus:

Pemphigus vulgaris may cause:

• Lesion that may extend deep into the skin
• Blisters that usually start in the mouth or on the scalp
• Lesions that progress to the face, neck, upper body, armpits, and groin
• Blisters which may appear in the esophagus, rectum, nose, throat, larynx (voice box), eyes, vulva, or rectum
• Red skin
• Painful, open sores
• Blisters that may expand into surrounding tissue when pressure is added to them
• An outer layer of skin to be easily rubbed off
• Soft and easily broken blisters that release fluid
• Large areas of open skin, increasing the risk of fluid imbalance and infection
• Blisters that usually heal without scarring, but may change skin color to brown

Pemphigus foliaceus may cause:

• Superficial lesions
• Blisters that itch or produce a burning sensation
• Sores that are usually not found in the mouth or on other mucus membranes
• Blisters that first show up on the face, scalp, chest, or upper back
• Open blisters, causing shallow sores
• Red skin
• Scales and crusts

Paraneoplastic pemphigus may cause:

• Sores on mucous membranes, in the mouth, eye, and esophagus
• Blisters that appear on palms of hands and soles of feet
• Itchy or painful lesions
• Severe lung problems

Diagnosis

You will be asked about your symptoms and medical history. A physical exam will be done. This will include a thorough skin exam. Special care is given to examining the lesions.

Tests to look for signs of an autoimmune disorder may include:

• Skin biopsies of the lesion and surrounding tissue
• Blood and skin tests

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Treatment

There is no cure for pemphigus, but treatment may help control your condition. If left untreated, pemphigus can lead to death.

Treatment aims to control the disease and prevent infection of the blistering lesions.

If a drug triggered pemphigus, the doctor will stop that medication. In some cases, stopping the drug is all that is needed for recovery.

Prevention

There are no current guidelines to prevent pemphigus because the cause is unknown.