Pemphigus is a rare autoimmune disease that causes painful blisters on the skin. There are three types, with pemphigus foliaceus being the least severe. Pemphigus vulgaris is the most common and can be fatal without treatment. Paraneoplastic pemphigus is the most dangerous and occurs in response to a tumor. Treatment involves the use of steroids and antibiotics. The disease is not contagious.
Pemphigus is a disease that affects the skin, causing painful bumps, scabs, and blisters. It is an autoimmune disease, which means that the body mounts an immune response and attacks normal skin cells. This condition has three types which vary in severity. All forms are quite rare, occurring in about one in 1 million people, except in the Mediterranean.
The most treatable and least severe type of pemphigus is pemphigus foliaceus. In this variant, a protein in the upper part of the skin is attacked by the immune system. This causes sores to form on the scalp and then, if left untreated, the body forms sores on the shoulder, face, chest and back. This type is characterized by its similarity in appearance to eczema and is often misdiagnosed as such.
Unlike other forms, the foliaceous is also much less painful. However, it still requires treatment as the blisters can cause disfigurement. The blisters can also be very itchy. While foliaceus is associated with the lowest mortality rates, any form of this condition requires treatment so it doesn’t become fatal.
The most common type of this condition is pemphigus vulgaris. It usually starts with sores and blisters forming in the mouth. Sores can also be present in the vocal cords and cause difficulty speaking. Also, blisters that form elsewhere on the body are extremely painful.
People of Jewish or Mediterranean descent seem more likely to get this form of the condition. It usually occurs when people are 40 or older, although some children can also get the condition. Without treatment, this condition is fatal in nearly all cases. With treatment the mortality rates dropped to 5-15%.
Paraneoplastic pemphigus is the least common but most dangerous form. It occurs in response to the presence of a tumor, whether benign or malignant. The lips, mouth, throat and body may have painful blisters and sores. If a tumor can be found and removed, this condition often resolves quickly. However, high mortality rates with this form are usually associated with cancerous tumors.
Treatment of pemphigus vulgaris and foliaceus focuses on the use of oral and sometimes topical steroids to stop the disease from progressing. Getting treatment is extremely important, as a serious infection usually occurs 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 smaller amounts. People with this condition usually also need antibiotics to help prevent or fight infections caused by the sores.
Pemphigus is diagnosed by laboratory analysis of samples of sores. It causes the skin to separate in quite obvious and unmistakable ways, so it can be observed and diagnosed quite easily.
This condition is not contagious in any of its forms. There may be a genetic component, 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 that is definitely not the case.
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