Pemphigus is a group of disorders that affects the skin. The attacks cause blisters and burn-like wounds on skin and mucous membranes (like mouth). There are 3 major forms of the disease: Pemphigus vulgaris—most common type of pemphigusPemphigus foliaceusParaneoplastic pemphigus—most serious type, usually occurs with
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.
Factors that may increase your chance of pemphigus include: Family members with pemphigus
A history of having autoimmune diseases, such as
systemic lupus erythematosus, or thymoma
Jewish or Mediterranean descent
Regular use of certain medications:
Chelating agents, such as penicillamineACE inhibitorsNonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirinAntibiotics, such as penicillinAntiseizures, such as phenobarbitalThiols
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 skinBlisters that usually start in the mouth or on the scalpLesions that progress to the face, neck, upper body, armpits, and groinBlisters which may appear in the esophagus, rectum, nose, throat, larynx (voice box), eyes, vulva, or rectumRed skinPainful, open soresBlisters that may expand into surrounding tissue when pressure is added to themAn outer layer of skin to be easily rubbed offSoft and easily broken blisters that release fluidLarge areas of open skin, increasing the risk of fluid imbalance and infectionBlisters that usually heal without scarring, but may change skin color to brown
Pemphigus foliaceus may cause: Superficial lesionsBlisters that itch or produce a burning sensationSores that are usually not found in the mouth or on other mucus membranesBlisters that first show up on the face, scalp, chest, or upper backOpen blisters, causing shallow soresRed skinScales and crustsWorsening symptoms in sunlight
Paraneoplastic pemphigus may cause: Sores on mucous membranes, in the mouth, eye, and esophagusBlisters that appear on palms of hands and soles of feetItchy or painful lesions
The doctor will ask 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:
of the lesion and surrounding tissue
Blood and skin tests
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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.
Wound care is important to prevent infections. The wounds are treated similarly to severe
burns. They will be cleaned and protected from further contamination. Antibiotic medications will also be applied to the wounds to prevent infections.
Medications can usually help control symptoms. However, these medications can also produce serious side effects. Your doctor may order blood and urine tests to check for adverse effects.
Medications to help control pemphigus include: Steroids—reduce swelling and calm the immune systemTopical steroids, including injection into specific lesionsImmunosuppressive medications—decrease the strength of your immune systemAntibiotics—treat or prevent infectionMedicated mouthwash—for pain reliefIV immunoglobulin—immune system elements from a healthy immune system, often given in combination a biologic antineoplastic agent
It takes some time for the immune system to clear. In fact, it may be several months or years for the skin sores to disappear. Some may have a complete remission after a period of medication treatment. Others will need to continue smaller doses of medication between outbreaks.
Sores in the mouth can make eating and dental care difficult. It is important to maintain good dental hygiene. If you cannot brush because of mouth sores, talk to your dentist. They can provide alternative ways to keep you mouth clean.
Lesions in the mouth can also make it difficult to eat. Soft diets may help until the lesions heal. Talk to a dietitian if you are having trouble getting adequate 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.
Good overall nutrition helps the body heal and fight disease.
Some foods may also make your 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 reaction in your skin. Read the labels of all foods to make sure they do not contain foods that can set off a reaction.
Paraneoplastic pemphigus may require the removal of a tumor. The removal may improve the disorder or decrease symptoms.
There are no currently guidelines to prevent pemphigus because the cause is unknown.
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Pemphigus. National Institute of Arthritis and Musculoskeletal and Skin Disease (NIAMS) website. Available at:http://www.niams.nih.gov/Health_Info/Pemphigus/default.asp#10. Updated June 2015. Accessed November 9, 2015.
Pemphigus vulgaris. Merck Manual Professional Version website. Available at: http://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-vulgaris. Updated June 2013. Accessed November 9, 2015.
Last reviewed November 2015 by Michael Woods, MD
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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