What is Pemphigus?

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Pemphigus is an illness that affects the skin, causing painful bumps, scabs and blistering to occur. It is an autoimmune disorder, which means the body produces an immune response and attacks normal skin cells. Pemphigus has three types that range in severity. All forms of the disease are quite rare, and except in the Mediterranean, statistically, pemphigus occurs in about one in a million people.

Pemphigus foliaceus is the most treatable and least severe type. In this variant, a protein at the top of the skin is attacked by the body’s immune system. This causes sores to form on the scalp, and then if untreated, the body forms sores on the shoulder, face, chest and the back. Pemphigus foliaceus is marked by its similarity in appearance to eczema, and is often misdiagnosed as such.

Unlike other forms of pemphigus, foliaceus is also much less painful. It still, however, requires treatment as the blisters can cause disfiguration. Blisters may also be very itchy. Although pemphigus foliaceus is associated with the lowest mortality rates, any form of pemphigus requires treatment so that the disease does not become fatal.

Pemphigus vulgaris is the most commonly occurring form. It usually begins with sores and blisters forming in the mouth. The sores can even be present in the vocal chords and cause difficulty talking. Additionally, the blisters that form elsewhere on the body are extremely painful.

People of Jewish or Mediterranean descent seem most likely to contract pemphigus vulgaris. It usually manifests when people are 40 or older, though some children may also get the condition. Without treatment pemphigus vulgaris was once fatal in nearly all cases. With treatment fatality rates have dropped to 5-15%.

Paraneoplastic pemphigus is least common but most dangerous. It occurs in response to the presence of a tumor, either benign or malignant. The lips, mouth, throat and body may all have painful blistering and sores. If a tumor can be found and removed, this form of pemphigus often resolves quickly. However, high mortality rates with this form are generally associated with tumors of cancerous origin.

Treatment of pemphigus vulgaris and foliaceus focuses on using oral and sometimes topical steroids to stop the course of the disease. It is extremely important that such treatment begin, as generally severe infection will occur on the skin, or in the bronchial tubes or lungs. Once the disease is under control, steroid doses will still continue but may be given in lower amounts. People with pemphigus also usually need antibiotics to help prevent or fight off infections caused by the sores.

Pemphigus is diagnosed by lab analysis of samples of the sores. Pemphigus causes the skin to separate in quite noticeable and unmistakable ways, thus it can be observed and diagnosed quite easily.

This condition is not contagious in any of its forms. There may be some genetic links to pemphigus, but one person cannot cause the disease in someone else. Because of the disfiguring sores associated with pemphigus many assume the condition must be contagious, but this is overwhelmingly not the case.

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Written by Tricia Ellis-Christensen

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