Autoimmune Skin Conditions

An autoimmune disease is a disorder in which the body is attacking itself. Normally, white blood cells produce antibodies that attack harmful cells as they appear in the body. The opposite happens in autoimmune diseases. Antibodies attack healthy tissues instead of the harmful ones. Chronic hives can be very uncomfortable and interfere with sleep and daily activities. For many people, antihistamines and anti-itch medications provide relief.

In pemphigus, autoantibodies react to antigens found on the surface of certain skin cells (keratinocytes). Keratinocytes are the major cell of the epidermis and they stick (adhere) together to form the barrier that is the epidermis and they serve as an anchor to the underlying skin layer (the dermis).
In pemphigoid, autoantibodies react against proteins found at the junction where the epidermis and dermis meet, known as the dermal-epidermal junction (DEJ). The DEJ is part of the basement membrane zone.
In linear IgA disease, autoantibodies react against structures found at the dermal-epidermal junction causing the epidermis and dermis to separate.
In dermatitis herpetiformis, it is believed that circulating IgA reacts against substances in the skin that ultimately results in damage to and the separation of the dermal-epidermal junction. However, in this disorder gluten sensitivity in combination with elevated levels of immunoglobulin A causes an immune system response, which may also contribute to the development of symptoms.
In epidermolysis bullosa acquisita, autoantibodies react to collagen VII, a protein that is essential in anchoring the basement membrane to the dermis.

Blood tests can reveal the characteristic antibodies associated with specific autoimmune blistering diseases.
A skin biopsy is a small sample of affected tissue that is taken and examined under a microscope, which may reveal characteristic findings.
Physicians also perform direct immunofluorescence (DIF) on a skin biopsy sample that presents a gold standard in the assessment of patients with bullous disorders. This is a test in which the sample is stained with special dyes that allow antibodies to seen under a special microscope.

Corticosteroids - The mainstay of treatment for autoimmune blistering diseases is treatment with corticosteroids such as prednisone. Corticosteroid therapy is not effective in all cases and long-term treatment with high-doses of corticosteroids can cause serious side effects.
Immunosuppressants - Additional drugs have been used to treat individuals with autoimmune skin diseases, either alone or in combination with corticosteroids. These drugs include drugs that suppress the immune system (immunosuppressive drugs) such as: mycophenolate, azathioprine or cyclophosphamide. Immunosuppressive biological therapies such as rituximab, and intravenous immunoglobulin G (IVIG). Plasmapheresis also is a well-established form of treatment for severe cases of autoimmune blistering diseases.