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Pemphigus foliaceus is an autoimmune disease that causes skin swelling and crusty lesions. It is caused by the immune system attacking a protein in the skin. Treatment includes drugs that suppress the immune system, but long-term steroid use has negative side effects. Other medications can also be used, and people with pemphigus are monitored for signs of other autoimmune diseases.
Pemphigus foliaceus is a rare autoimmune disease that affects the mucous membranes and skin. People with this disease have skin that swells easily and often develop crusty, scaly lesions on the skin on the scalp, face, chest, and back. Anyone of any age can develop this disease, but it most often develops in people over the age of 50. Pemphigus foliaceus can be treated with drugs that suppress the immune system.
Normally, a healthy immune system is only sensitized to proteins of foreign origin, such as those of bacteria and viruses. Autoimmune disease develops when the immune system becomes responsive to a protein made by the body. In the case of pemphigus foliaceus, this protein is desmoglein 1, which is produced by cells in the top layer of skin, called the epidermis. There are two other types of pemphigus disease, in which the immune system becomes sensitized to other desmoglein proteins.
The first symptom of pemphigus foliaceus is usually the development of blisters on the chest and back. The blisters can spread to other parts of the body and develop into crusty, scaly sores as they heal. People with this skin disorder are usually in good overall health, but tend to have blistering episodes that last several months or years. Spontaneous remission of the disease may occur, leading to healing of the current skin lesions without new ones appearing. Even after remission, however, the disease can reappear at any time.
This is an unpleasant disease, but it is not serious or life-threatening because desmoglein 1 is present only in some cells of the epidermis. Therefore, only the upper layers of the skin are ever affected by this disease. Symptoms can be treated with drugs that suppress the immune system such as corticosteroids, but because long-term steroid use has negative side effects such as an increased risk of osteoporosis and cataracts, corticosteroid treatment courses are usually short.
Other types of medications besides corticosteroids can also be used to treat pemphigus foliaceus. Topical antibiotic creams, antimalarial medications, and a drug called nicotinamide can be used to reduce the risk of infection, reduce skin inflammation, and help lesions heal. Some people with pemphigus benefit from high-level UV protection, because sun exposure can trigger the development of skin lesions.
Autoimmune skin diseases such as pemphigus often occur in association with other types of autoimmune diseases. The most likely diseases with pemphigus are thymoma, myasthenia gravis and lupus erythematosus. Therefore, people with pemphigus are often closely monitored for signs of other emerging autoimmune diseases.
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