Pemphigus

Causes

Pemphigus is an autoimmune disorder. The immune system attacks healthy skin and mucus membranes. The attack causes 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, medicine may be the cause.

Risk Factors

Pemphigus is more common in people of Jewish or Mediterranean descent. Other things that may raise the risk 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 some medicines:
  • 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:

• Lesions that may extend deep into the skin
• Blisters that often start in the mouth or on the scalp
• Lesions that progress to the face, neck, upper body, armpits, and groin
• Blisters that may form 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 that is easily rubbed off
• Soft and easily broken blisters that release fluid
• Large areas of open skin, which raises the risk of fluid imbalance and infection
• Blisters that often heal without scarring, but may change skin color to brown

Pemphigus foliaceus may cause:

• Lesions that are on the top of the skin
• Blisters that itch or produce a burning sensation
• Sores that are often 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

IgA pemphigus may cause:

• Skin lesions that appear on the trunk and upper body
• Lesions do not often form in places like the nose, mouth or eyes

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

The doctor will ask about symptoms and health 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

Skin Biopsy

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Treatment

The goal of treatment is to manage symptoms and prevent the blistering lesions from getting infected. There is no cure for pemphigus. If left untreated, pemphigus can lead to death.

If a drug triggered pemphigus, the doctor will stop that medicine. For some stopping the drug is all that is needed for recovery.

Wound Care

Wound care is key to prevent infections. The wounds are treated similarly to severe burns. They will be cleaned and protected from more contamination. Antibiotics will also be put on the wounds to prevent infections.

Medication

Medicines can often help control symptoms, but they can have some serious side effects. Blood or urine tests may be needed to check for side effects.

Medicines may be given to:

• Reduce swelling and calm the immune system
• Make the immune system weaker
• Ease pain
• Treat or prevent infection

It takes some time for the immune system to clear. It may take months or even years for the skin sores to go away. Some people may have no further problems after taking medicine for a time. Others will need to keep taking smaller doses of medicine between outbreaks.

Mouth Care

Sores in the mouth can make eating and dental care difficult. It is important to have good dental hygiene. Talk to the dentist if brushing is too painful due to the mouth sores. There are other ways to help keep the mouth clean.

Lesions in the mouth can also make it hard to eat. Soft diets may help until the lesions heal. Talk to a dietitian about getting proper nutrition with a soft diet.

Avoid foods that can cause irritation in your mouth. This includes foods that are high in acids, spicy, or hard.

Lifestyle Changes

Good overall nutrition helps the body heal and fight disease.

Some foods may also make symptoms worse. They may also trigger the onset of more sores. Foods that have been linked to these problems include garlic, onions, and leeks. Keep track of the foods that seem to cause a skin reaction. Read the labels of all foods to make sure they do not have foods that can set off a reaction.

Surgery

Paraneoplastic pemphigus may require the removal of a tumor. The removal may improve the disorder or decrease symptoms.

Prevention

There are no current guidelines to prevent pemphigus.