Pemphigus: Diagnosis, Treatment, and Steps to Take

Diagnosis of Pemphigus

Early diagnosis is important, so if you have blisters on the skin or in the mouth that do not go away, it is important to see a doctor as soon as you can. Your doctor may try to rule out other conditions first, since pemphigus is a rare disease. Your doctor may:

• Take your medical history, and give you a physical exam. A dermatologist (a doctor who specializes in conditions of the skin, hair, and nails) may ask you about your medical history and look at the appearance and location of blisters. He or she may run a finger or cotton swab over the surface of your skin to see if it shears off easily.
• Take a tissue sample. Your doctor may take a sample from one of your blisters to:
  • Examine it under the microscope to look for cell separation and to determine the layer of skin in which the cells are separated.
  • Determine which antibodies attacked the skin.
• Take a blood sample. Blood tests can help determine the types of antibodies that are in the blood and their levels, which can help predict the severity of the disease. This blood test may also be used later on to see if treatment is working.

Treatment of Pemphigus

There is no cure for pemphigus, but treatment can control the disease in most people. The initial goal of treatment is to clear existing blisters and help prevent relapses. Treatment typically depends on the severity and stage of the disease.

Symptoms of pemphigus may go away after many years of treatment, but most people need to continue taking medications to keep the disease under control. Treatment for pemphigus may involve the following medications:

• Corticosteroids. These anti-inflammatory medicines are a mainstay of treatment for pemphigus. They may be applied topically as a cream or ointment, or by mouth or injection (systemically). Most people will be prescribed systemic corticosteroids, at least initially, to bring the disease under control. Because they are potent drugs, your doctor will prescribe the lowest dose possible to achieve the desired benefit.
• Biologic response modifiers (or biologics). These target specific immune messages and interrupt the signal, helping to stop the immune system from attacking the skin. Rituximab is an approved biologic administered directly in the vein. It targets and depletes the immune cells that ultimately make the disease-causing antibodies.
• Antibiotics, antivirals, and antifungal medications to control or prevent infections.

If the above treatments do not work or are not tolerated, other treatments may be considered. These treatments include:

• Immunosuppressants. Although less effective than rituximab, these are oral medications that help suppress or curb the overactive immune system and may help to lower the dose of daily steroid.
• Plasmapheresis or immunoadsorption. These are procedures that remove or dilute out damaging antibodies from the blood.
• Intravenous immunoglobulin therapy. This is an intravenous infusion of pooled antibodies from 1,000 or more healthy blood donors, which dilute out the bad antibodies and calm inflammation.

Be sure to report any problems or side effects from medications to your doctor.

In some cases, a person with pemphigus may need to be hospitalized to treat health problems that the disease or its treatment can cause. Widespread sores on the skin can result in dehydration or infection, and painful blisters in the mouth can make it difficult to eat. In the hospital, you may be given an IV to replace lost fluids, to get much-needed nutrition, and to treat infection.

Who Treats Pemphigus?

The following health care providers may diagnose and treat pemphigus:

• Dermatologists, who specialize in conditions of the skin, hair, and nails.
• Dentists, who can tell you how to take care of your gums and teeth if you have blisters in your mouth.
• Mental health professionals, who help people cope with difficulties in the home and workplace that may result from their medical conditions.
• Ophthalmologists, in cases where the eyes are affected. Ophthalmologists specialize in treating disorders and diseases of the eye.
• Otolaryngologists, if the larynx (voice box) or upper throat is affected and visualization is necessary to ensure symptoms are due to blisters or some other factor.
• Primary care doctors, such as a family physician or internal medicine specialist, who coordinate care between the different health care providers and treat other problems as they arise.