Pemphigus

What is Pemphigus?

Pemphigus refers to a group of rare autoimmune skin disorders that cause sores and blisters on the mucous membranes or the skin. Areas that can be affected include the genitals, throat, nose, eyes, and mouth.

There are different types of pemphigus, but the two most common are pemphigus foliaceus and pemphigus vulgaris. While pemphigus foliaceus will affect your skin and typically be itchy rather than painful, pemphigus vulgaris will characteristically start in the mouth.

This disorder affects all ages, but it is most common amongst individuals who are middle aged and older. It is usually chronic, but it can be controlled with the help of an early diagnosis and the right treatment.

What are the Symptoms of Pemphigus?

The development of blisters on the mucous membranes and skin is the main sign of pemphigus. These blisters could rupture easily, and they could leave open sores behind. Those sores could then get infected and they might ooze.

In pemphigus vulgaris, you will notice blisters in the mouth and later on the skin or genitals. The blisters won’t itch, but they’ll be painful, and the ones that are in the throat or mouth could make it difficult to eat or swallow.

In pemphigus foliaceus, the blisters typically won’t be painful because they’ll be on the skin rather than on the mucous membranes. Most of the blisters will be on the shoulders, back, and chest. The skin could become itchy and crusty.

Pemphigus Causes

Pemphigus is caused by an abnormal reaction of the immune system which causes it to attack healthy cells on the top layers of skin and the mucous membranes. In this case, it creates antibodies which attempt to fight desmogleins, a type of protein found in the skin which work to hold skin cells together.

When desmogleins are attacked, the skin cells are forced apart. Fluid begins to collect in between layers of skin, which is what creates the chronic blisters associated with pemphigus.

It isn’t clear why this abnormal immune system response occurs. Although the condition is not hereditary, it appears that genetics could play a certain role in the condition because it appears to be more common in certain ethnic groups. For example, people with Mediterranean or European Jewish heritage are at increased risk of the condition.

However, while there could be genetic traits which could make someone more likely to develop pemphigus, it’s possible that it only occurs after exposure to certain environmental triggers, such as certain materials or chemicals. Pemphigus can also occur after the use of certain medications such as certain drugs used to treat high blood pressure. However, in these instances, the condition usually goes away after stopping the medication.

How is Pemphigus Treated?

Treatment for pemphigus might include therapies and medications that are similar to those that would be used to treat patients with severe burns.

Your doctor might prescribe anti-inflammatory medications, such as corticosteroids, at high doses in order to control your immune system. Corticosteroid cream might also be prescribed and applied to blisters. Antibiotics and immunosuppressants might also be used to treat pemphigus.

Severe cases might require a hospital stay.

Pemphigus Prevention

Since the causes of pemphigus aren’t completely understood, there is no known way to prevent the condition. However, it is possible to manage pemphigus successfully and prevent secondary infections which could occur as a result of the condition.

First of all, be sure to carefully follow your health care provider’s instructions for wound care, and always wash hands thoroughly before touching blisters. You should also take the medicines prescribed to you until your doctor tells you to stop, even if your skin appears to have improved, as stopping early could cause the condition to worsen.

Wash clothes, bed sheets, and towels regularly to minimize the risk of bacteria entering blisters. You shouldn’t share these items with other people.

Avoid contact sports or other activities which could cause irritate blisters. You should also take care to stay out of the sun as this could make existing blisters worse or trigger new ones.

If you have blisters inside your mouth, avoid strong-tasting or irritating foods, such as onion, garlic, and chili. Try to consume only soft foods rather than abrasive ones which could trigger new blisters.