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. This condition has three types that range in severity. All forms are quite rare, occurring in about one in 1 million people, except in the Mediterranean.
The most treatable, least severe type of pemphigus is pemphigus foliaceus. 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. This type is marked by its similarity in appearance to eczema, and is often misdiagnosed as such.
Unlike other forms, foliaceus is also much less painful. It still, however, requires treatment as the blisters can cause disfiguration. Blisters may also be very itchy. Although foliaceus is associated with the lowest mortality rates, any form of this condition requires treatment so that it does not become fatal.
The most commonly occurring type of this condition is pemphigus vulgaris. 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 this form of the condition. It usually manifests when people are 40 or older, though some children may also get the condition. Without treatment, this condition is fatal in nearly all cases. With treatment fatality rates have dropped to 5-15%.
Paraneoplastic pemphigus is least common, but most dangerous form. 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 condition 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. Getting treatment is extremely important, as a severe infection will generally 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 this condition 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. It 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 a genetic component to it, 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.
