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Pemphigus
(Pemphigus Syndromes)

Definition:
Pemphigus is a group of rare autoimmune disorders that causes large skin blisters. The immune system normally protects against germs and other foreign invaders. Pemphigus occurs when your immune system mistakenly attacks your own skin and mucous membranes. There are three forms of the disease:
  • Pemphigus vulgaris–the most common type. It typically involves the mouth, scalp, face, neck, armpits, or trunk. The lesions may extend deep into the layers of the skin.
  • Pemphigus foliaceus–produces a more superficial lesion.
  • Paraneoplastic pemphigus–the most serious type. It usually occurs in someone who has cancer.
Causes:
With pemphigus, the immune system produces antibodies that cause the skin and mucous membranes to break out in blisters and burn-like sores. What causes the body to attack itself is not known. Sometimes a drug can precipitate symptoms.

Risk Factors:
A risk factor is something that increases your chances of getting a disease or condition. Risk factors for pemphigus include:
  • Family members with pemphigus
  • A history of having autoimmune diseases, such as myasthenia gravis, lupus, or thymoma
  • Age: Middle to late adulthood
  • Jewish or Mediterranean descent
  • Regular use of certain drugs, including:
    • Penicillamine
    • Captopril
    • Rifampin
    • Piroxicam
    • Penicillin
    • Phenobarbital
Symptoms:
Itching and pain are common symptoms. Pemphigus may occur over a small or large section of the skin. Symptoms differ among the types of pemphigus.

Pemphigus Vulgaris
  • Blisters usually start in the mouth or on the scalp.
  • Lesions progress to the face, neck, upper body, armpits, and groin.
  • Blisters also may occur in the esophagus, rectum, nose, throat, larynx (voice box), eyes, vulva, or rectum.
  • Surrounding skin may be red or normal color.
  • Pressure on the blister may cause it to expand into surrounding tissue.
  • Outer layer of skin can easily be rubbed off.
  • Blisters are soft and break easily, releasing fluid.
  • Open sores are painful.
  • Large areas of skin may open up, increasing the risk of fluid imbalance and infection.
  • Blisters usually heal without scarring, but skin color may change to brown.
Pemphigus Foliaceus
  • Blisters are itchy or produce a burning sensation.
  • Sores are usually not found in the mouth or on other mucous membranes.
  • Blisters usually first show up on the face, scalp, chest, or upper back.
  • Blisters open, causing shallow sores.
  • Skin is red.
  • Scales and crusts form.
  • Sunlight may make symptoms worse.
Paraneoplastic Pemphigus
  • Sores occur on the mucous membranes, in the mouth, eye, and esophagus.
  • Blisters often appear on palms of hands and soles of feet.
  • Lesions are itchy or painful.
Diagnosis:
The doctor will ask about your symptoms and medical history and perform a physical exam, with special care given to examining the lesions. This will include a thorough skin exam.

Tests may include:

  • Skin biopsies of the lesion and surrounding tissue
  • Skin test to determine the level of antibodies by immunofluorescence
  • Blood test to check the level of antibodies by immunofluorescence
Treatment:
There is no cure for pemphigus. Treatment aims to control the disease and prevent infection of the blistering lesions. The wounds are treated similarly to severe burns, with antibiotics and petroleum jelly-impregnated bandages. Earlier treatment produces better results. Even with treatment, a series of remissions and relapses usually occurs. If left untreated, pemphigus can lead to death.

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

Medication
Drugs can usually help control symptoms. Since the medications can produce serious side effects, your doctor may order periodic blood and urine tests to check for adverse effects.

Drugs that may be given include the following :

  • Oral steroids, such as Prednisone
  • Topical steroids, including injection into specific lesions
  • Immunosuppressive drugs (drugs that decrease your immune system’s ability to function), such as:
    • Azathioprine (Imuran)
    • Cyclophosphamide (Cytoxan)
    • Methotrexate
    • Gold
    • Cyclosporine
  • Antimalarial drugs such as hydroxychloroquine (Plaquenil)
  • A combination of nicotinamide and tetracycline
  • Antibiotics (topical or oral)–to treat any bacterial skin infections that occur
  • Intravenous immunoglobulin
  • Viscous lidocaine mouthwash may numb the mouth and provide pain relief
Plasmapheresis
For this procedure, whole blood is removed and the plasma is separated by centrifugation. Then the packed cells suspended in saline are reinjected. This is used in certain cases of pemphigus in combination with immunosuppressive drugs.

Lifestyle Changes
Diet–good nutrition helps the body heal and fight disease. Also, some foods may make your symptoms worse or trigger the onset of more sores. Some patients notice they are sensitive to garlic, onions, and leeks. Keep track of the foods that seem to cause a reaction in your skin and avoid these foods. Read the labels of all foods to make sure they do not contain small amounts of foods that can set off a reaction. Soft diets may be necessary if you have oral lesions.

Sun Exposure–sunlight may worsen symptoms of pemphigus foliaceus. If you have this type of the disease you should avoid sun exposure.

Surgery
In some cases of paraneoplastic pemphigus, surgical removal of the tumor may improve the disorder or decrease symptoms.

Prevention:
There are no guidelines for preventing pemphigus because the cause is unknown.
 
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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