Pemphigus Vulgaris

Pemphigus vulgaris, also known under the acronym PV, is one of the mysterious autoimmune diseases. It affects the skin and mucous membranes located in the mouth. The immune system of the body sends antibodies to attack healthy tissues in these areas by mistake. They destroy the binding between cells, causing blisters to appear. These blisters heal very slowly and can expand to cover entire areas of the body. Sometimes, the blisters only occur inside the mouth and the majority of patients report the first symptoms in there.

A distinctive trait of pemphigus vulgaris is the large number of blisters and their very slow rate of healing. Weeks or even months are needed and sometimes the blisters cause itching, burning, stings or severe pain. In the most severe cases, the ulcers can lead to horrible scars, strong infection or even death due to a loss of fluids. If treatment is applied in time, fatalities from the disease are extremely rare today.

Like most autoimmune diseases, pemphigus vulgaris is poorly understood. In addition, it is very rare and most doctors are not familiar with it, this is why most cases are not properly diagnosed for up to half a year. Effective treatment for the condition is available today but between 2 and 5 years are needed until remission starts. Modern treatment also greatly reduces the risk of scarring.

What are the symptoms of pemphigus vulgaris?

In most cases, the first blisters emerge inside the mouth. After several weeks or even months, they expand and start appearing on the skin as well. The condition tends to be cyclical, with periods of serious blister outbreaks alternating with time of remission, when they heal completely. This behaviour is unpredictable and scientists are not sure what causes it.

The lesions located inside the mouth can be extremely painful and uncomfortable. They cause eating, brushing the teeth and even speaking to be very painful. Blisters can also affect the voice, if they reach the larynx.

On the skin, these blisters sometimes expand in size and unite into a large area of raw skin, which looks like an open wound. After a while, the area is covered by crusts and scabs. Permanent skin discoloration is possible but more serious scars are rare.

What causes pemphigus vulgaris?

Pemphigus vulgaris is part of the autoimmune diseases group. Due to unknown reasons, the body's immune system becomes confused and starts to attack its own healthy tissues, instead of pathogens.

Autoimmune diseases can affect various parts of the body. Pemphigus vulgaris is an antibody attack on the cells in the secondary layers of skin, as well as mucous membranes. These membranes are found in the mouth, throat and nose, as well as the anus and genitals. Blisters appear in the area, as the links between cells are destroyed.

All autoimmune diseases remain a mystery for scientists and pemphigus vulgaris is no exception. Nobody knows what causes the condition. While some genes that appear to increase the risk have been identified, the disease is not transmitted in the family.

Treatment options

While the disease itself can't be treated, the symptoms themselves can be reduced and there are a number of drugs available that can decrease the strength of the immune attack.

Steroid drugs, like corticosteroids, can be effective in large doses. The steroids have to be administered for a long period, of several weeks and months. They speed up the healing rate of the blisters and prevent the formation of new ones. Steroids are known to cause serious side effects. For this reason, the treatment is combined with medicines that suppress the immune system, in order to reduce its duration. The steroid dosage is also reduced in time.

In some cases, one cure is enough to heal pemphigus vulgaris completely. While some patients need to continue the treatment in order to prevent a new outbreak of the pemphigus vulgaris, others can be healed completely.

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