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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 mucus membranes. There are three forms of the disease:
Pemphigus vulgarisis the most common type of pemphigus. It typically involves the mouth, scalp, face, neck, armpits, or trunk. The lesions may extend deep into the layers of the skin.
Pemphigus foliaceusproduces more superficial lesions.
Paraneoplastic pemphigusis 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 mucus 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 chance 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 VulgarisBlisters usually start in the mouth or on the scalpLesions progress to the face, neck, upper body, armpits, and groinBlisters also may occur in the esophagus, rectum, nose, throat, larynx (voice box), eyes, vulva, or rectumSurrounding skin may be red or normal colorPressure on the blister may cause it to expand into surrounding tissueOuter layer of skin can easily be rubbed offBlisters are soft and break easily, releasing fluidOpen sores are painfulLarge areas of skin may open up, increasing the risk of fluid imbalance and infectionBlisters usually heal without scarring, but skin color may change to brown Pemphigus FoliaceusBlisters are itchy or produce a burning sensationSores are usually not found in the mouth or on other mucus membranesBlisters usually first show up on the face, scalp, chest, or upper backBlisters open, causing shallow soresSkin is redScales and crusts formSunlight may make symptoms worse Paraneoplastic PemphigusSores occur on the mucous membranes, in the mouth, eye, and esophagusBlisters often appear on palms of hands and soles of feetLesions are itchy or painful DiagnosisThe 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 immunofluorescenceBlood test to check the level of antibodies by immunofluorescence Skin Biopsy 2008 Nucleus Medical Art, Inc. 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. MedicationDrugs 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 PrednisoneTopical steroids, including injection into specific lesions Immunosuppressive drugs (drugs that decrease your immune systems ability to function), such as: Azathioprine (Imuran)Mycophenylate MofetilCyclophosphamide (Cytoxan)MethotrexateGoldCyclosporineAntimalarial drugs such as hydroxychloroquine (Plaquenil)A combination of nicotinamide and tetracyclineAntibiotics (topical or oral) to treat any bacterial skin infections that occurIntravenous immunoglobulinViscous lidocaine mouthwash may numb the mouth and provide pain reliefSucralfate mouthwash may coat and soothe the mouth and provide pain relief PlasmapheresisFor this procedure, whole blood is removed and the plasma is separated by centrifugation. The packed cells are then suspended in saline are reinjected. This is used in certain cases of pemphigus in combination with immunosuppressive drugs.
Lifestyle ChangesDietGood 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. SurgeryIn some cases of paraneoplastic pemphigus, surgical removal of the tumor may improve the disorder or decrease symptoms. PreventionThere are no guidelines for preventing pemphigus because the cause is unknown. RESOURCES: American Autoimmune Related Diseases Association, Inc.http://www.aarda.org International Pemphigus Foundationhttp://www.pemphigus.org CANADIAN RESOURCE: Canadian Dermatology Associationhttp://www.dermatology.ca/index.html Health Canadahttp://www.hc-sc.gc.ca/index_e.html References: Beers MH et al. The Merck Manual of Medical InformationHome Edition . 2nd ed. Simon and Schuster, Inc.; 2003. Cecil Textbook of Medicine . 21st ed. WB Saunders Co.; 2000. Chams-Davatchi C, Esmaili N, Daneshpazhooh M, Valikhani M, Balighi K, Hallaji Z, Barzegari M, Akhyani M, Ghodsi SZ, Seirafi H, Nazemi MJ, Mortazavi H, Mirshams-Shahshahani M. Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris. J Am Acad Dermatol . 2007 Oct;57(4):622-8.
Clinical Dermatology . 3rd ed. Mosby-Year Book Inc.; 1996. Conn's Current Therapy 2001 . 53rd ed. WB Saunders Co.; 2001. Diaz LA. Rituximab and pemphigusa therapeutic advance. N Engl J Med . 2007 Aug 9;357(6):605-7. Ferri FF. Ferri's Clinical Advisor: Instant Diagnosis and Treatment . 8th ed., St. Louis: Mosby; 2006. Goldman L. Cecil Textbook of Medicine . 22nd ed. Philadelphia: Saunders; 2004. Griffith's 5-Minute Clinical Consult . 2001 ed. Lippincott Williams & Wilkins; 2001. Harrison's Principles of Internal Medicine . 14th ed. The McGraw-Hill Companies; 2000. Textbook of Primary Care Medicine . 3rd ed. Mosby Inc.; 2001. Last reviewed November 2007 by Ross Zeltser, MDPlease 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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