Learn about symptoms, causes, treatment and self-care tips for this group of rare conditions that causes blisters and sores on the skin or mucous membranes.
Pemphigus is a disease that causes blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals.
Pemphigus can occur at any age, but it's most often seen in people who are middle-aged or older. It tends to be a long-lasting (chronic) condition, and some types can be life-threatening without treatment. Treatment with medication usually controls it.
Pemphigus is a rare skin disorder characterized by blistering of your skin and mucous membranes. The most common type is pemphigus vulgaris, which involves painful sores and blisters on your skin and in your mouth.
Pemphigus causes blisters on your skin and mucous membranes. The blisters rupture easily, leaving open sores, which may ooze and become infected.
The signs and symptoms of two common types of pemphigus are as follows:
Pemphigus is distinct from bullous pemphigoid, which is a blistering skin condition that affects older adults and may cause death.
See your doctor if you have blisters inside your mouth or on your skin that don't heal.
Pemphigus is an autoimmune disorder. Normally, your immune system produces antibodies to fight off harmful invaders, such viruses and bacteria. But in pemphigus, the body produces antibodies that damage cells of your skin and mucous membranes.
Pemphigus isn't contagious. In most cases, it's unknown what triggers the disease.
Rarely, pemphigus is triggered by the use of angiotensin-converting enzyme inhibitors, penicillamine and other drugs.
Your risk of pemphigus increases if you're middle-aged or older. The condition tends to be more common in people of Middle Eastern or Jewish descent.
Possible complications of pemphigus include:
Blisters occur with a number of more common conditions, so pemphigus, which is rare, can be difficult to diagnose. Your doctor may refer you to a specialist in skin conditions (dermatologist).
Your doctor will talk with you about your medical history and examine your skin and mouth. In addition, you may undergo tests, including:
Treatment usually begins with medications that are intended to suppress blister formation. It's generally more effective when it begins as early as possible. If use of a drug triggered your condition, stopping use of it may be enough to clear up your pemphigus.
The following prescription medications may be used alone or in combination, depending on the type and severity of your pemphigus and whether you have other medical conditions:
Corticosteroids. For people with mild disease, corticosteroid cream may be enough to control it. For others, the mainstay of treatment is an oral corticosteroid, such as prednisone pills.
Using corticosteroids for a long time or in high doses may cause serious side effects, including diabetes, bone loss, an increased risk of infection, stomach ulcers and a redistribution of body fat, leading to a round face (moon face).
Many people get better with treatment, although it may take years. Others need to take a lower dose of medication indefinitely to prevent their signs and symptoms from returning. And some people need treatment in a hospital — for example, to care for severe or infected sores.
Here are steps you can take to improve your skin and overall health:
Pemphigus may be difficult to live with, especially if it affects your daily activities or causes lost sleep or stress. You may find it helpful to talk to others with the disease. You can find in-person or online support groups. Ask your doctor for suggestions.
You're likely to first see your primary care doctor. He or she may refer you to a doctor who specializes in skin disorders (dermatologist).
Here's some information to help you get ready for your appointment.
Before your appointment, make a list of:
For pemphigus, some basic questions to ask your doctor include:
Your doctor is likely to ask you a number of questions, such as: