Pemphigus foliaceus is a rare autoimmune disease that affects the mucous membranes and the skin. People with this disease have skin that blisters easily, and they often develop crusty, scaly lesions on the skin of the scalp, face, chest and back. Anyone of any age can develop this disease, but it most often develops in people who are more than 50 years old. Pemphigus foliaceus can be treated with medications that suppress the immune system.
Normally, a healthy immune system is sensitized only to proteins of foreign origin, such as those of bacteria and viruses. Autoimmune disease develops when the immune system becomes reactive to a protein produced by the body. In the case of pemphigus foliaceus, this protein is desmoglein 1, produced by cells in the top layer of skin, called the epidermis. Two other types of pemphigus disease exist, 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 disease usually are in good general health but tend to have episodes of skin blisters that last several months or years. Spontaneous remission of the disease can occur, leading to healing of current skin lesions without new ones appearing. Even after remission occurs, however, the disease can reappear at any time.
This is an unpleasant disease, but it is not serious or life-threatening, because the desmoglein 1 is present only in certain cells of the epidermis. Therefore, only the top layers of skin are ever affected by this disease. Symptoms can be treated with immune system-suppressing medications such as corticosteroids, but because long-term steroid use has negative side effects such as increased risk of osteoporosis and cataracts, courses of corticosteroid treatment generally are short.
Other types of medication in addition to corticosteroids also can be used to treat pemphigus foliaceus. Topical antibiotic creams, anti-malarial drugs and a medication called nicotinamide can be used to reduce infection risks, 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 disorders such as pemphigus often occur in association with other types of autoimmune disease. The diseases most likely to occur with pemphigus are thymoma, myasthenia gravis and lupus erythematosus. Therefore, people with pemphigus are often closely monitored for signs of other emerging autoimmune diseases.
