Why is pemphigus vulgaris fatal

Antibiotics may be needed to treat infections in ruptured blisters. Dressings can protect raw, oozing areas. High doses of corticosteroids are the mainstay of treatment.

They are taken by mouth orally or, if the person is hospitalized, may be given by vein intravenously. If the disease is controlled, the dose of corticosteroids is gradually reduced tapered. Sometimes rituximab , a drug that suppresses the immune system immunosuppressant , is given with corticosteroids.

If the person does not respond to treatment or the disease flares up as the dose is tapered, an immunosuppressant, such as azathioprine , cyclophosphamide , methotrexate , mycophenolate mofetil, cyclosporine , or rituximab if not already given , is also given. Immunosuppressants may also be given to reduce the need for corticosteroids in people who have needed corticosteroids for a long time or at high doses. Immune globulin given by vein is another treatment that can be used for severe pemphigus vulgaris.

Some people respond well enough to discontinue drug therapy, whereas others must continue taking low doses of the drugs for long periods. People with severe pemphigus vulgaris may also undergo plasma exchange Plasma exchange In apheresis, blood is removed from a person and then returned after substances are removed from it. Apheresis can be used to Obtain healthy blood components from a donor to transfuse to a person The following is an English-language resource that may be useful.

National Organization for Rare Disorders: Information about pemphigus, including links to resources and supporting organizations. Merck and Co. From developing new therapies that treat and prevent disease to helping people in need, we are committed to improving health and well-being around the world. The Manual was first published in as a service to the community. Learn more about our commitment to Global Medical Knowledge. This site complies with the HONcode standard for trustworthy health information: verify here.

Common Health Topics. More Information. Blistering Diseases. Test your knowledge. Atopic dermatitis, commonly known as eczema, is a chronic condition that causes itchy inflammation of the upper layers of skin. Eczema is especially common in people who have hay fever or asthma, and in people whose family members have these conditions.

Eczema is common, particularly in urban areas and developed countries. Pemphigus vulgaris is the most common type of a group of autoimmune disorders called pemphigus. Each type of pemphigus is characterized by where the blisters form. This disease usually starts with blisters in the mouth and then on the skin. The blisters sometimes affect the membranes of the genitals. Pemphigus vulgaris can be dangerous. Treatment is essential, and typically involves the use of corticosteroids to suppress the immune system.

Some of these complications can be fatal. The death rate from this disease had averaged 75 percent before corticosteroids were introduced in the s. The immune system produces proteins called antibodies. Antibodies normally attack harmful foreign substances like bacteria and viruses. Pemphigus vulgaris occurs when the immune system mistakenly makes antibodies against proteins in healthy skin and mucous membranes. The antibodies break down the bonds between the cells, and fluid collects between the layers of the skin.

This leads to blisters and erosions on the skin. Pemphigus vulgaris can affect people of all races, genders, and ages. However, the condition is more common in the following groups:. A dermatologist will conduct a physical examination of your skin blisters. Your doctor may then take a biopsy of the blister, which involves removing a piece of tissue for analysis, and looking at it under a microscope to confirm the diagnosis.

The biopsy may be treated in the laboratory with chemical substances that help your doctor find the abnormal antibodies. Your doctor can use this information to determine the type of pemphigus.

The different types of pemphigus are diagnosed based on the location of the blisters. They include:. Pemphigus vulgaris is the most common type of pemphigus in the Unites States. Blisters usually first appear in the mouth. They can be painful. Blisters may then appear on the skin and sometimes on the genitals. The blisters first appear on the face and scalp. Blisters then appear on the chest and back. The blisters are usually itchy and painless. A very rare type of pemphigus that occurs in people with some cancers is called paraneoplastic pemphigus.

The blisters and sores may appear in the mouth, on the lips, and on the skin. This type may also cause scars on the eyelids and eyes. It can also cause lung problems. Treatment is aimed at reducing pain and symptoms and preventing complications like infection. The blisters are fragile and can easily burst open, leaving areas of raw unhealed skin that are very painful and can put you at risk of infections.

There's currently no cure for pemphigus vulgaris, but treatment can help keep the symptoms under control. The condition can affect people of all ages, including children, but most cases develop in older adults between the ages of 50 and It isn't contagious and can't be passed from one person to another. The blisters usually develop in the mouth first, before affecting the skin a few weeks or months later.

There may be times when the blisters are severe flare-ups , followed by periods when they heal and fade remission. It's impossible to predict when this might happen and how severe the flare-ups will be. Blisters in the mouth often turn into painful sores, which can make eating, drinking and brushing teeth very difficult. The voice can become hoarse if the blisters spread to the voice box larynx. Sores on the skin can join together to form large areas of painful, raw-looking skin, before crusting over and forming scabs.

They don't usually leave any scars , although affected skin can occasionally become permanently discoloured. As well as getting blisters in the mouth, they can also develop in other areas of the digestive system's soft tissue lining, including the nose, throat, anus, genitals and vagina. The thin membrane that covers the front of the eye and inside of the eyelids conjunctiva can also be affected.