Pemphigoid
Understanding Pemphigoid
Pemphigoid is a group of rare autoimmune skin conditions that cause large fluid filled blisters to form. An autoimmune disorder occurs when the immune system malfunctions and creates antibodies that attack and cause damage to a part of the body. In pemphigoid, the skin, and more specifically the basement membrane below the epidermis, is attacked. This results in blisters that form on the skin and mucous membranes (mucus-secreting tissue such as those lining the mouth and nose). Although pemphigoid can occur at any age and in any race and gender, it occurs more commonly in the elderly. Pemphigoid is similar to pemphigus, another group of rare autoimmune blistering disorders.
Symptoms of Pemphigoid
Symptoms of pemphigoid, including location and nature of blisters, and treatments depend on the type of pemphigoid. There are several types:
Cicatricial pemphigoid is also known as mucous membrane pemphigoid, as the lesions usually occur on mucous membrane surfaces. The gums are commonly affected, and epithelium (thin layer of protective tissue) of the eye (cornea and conjunctiva) is affected in up to one-third of cases. Cicatricial pemphigoid can result in blindness if it involves the eyes, respiratory compromise if it involves the deeper parts of the throat, and infections if it leaves areas of open skin.
Bullous Pemphigoid is limited to the skin with blisters presenting primarily on the abdomen, groin, back, arms and legs. The blisters may itch and be painful.
Diagnosis of Pemphigoid
Diagnosing pemphigoid can be very difficult and may take months or even years. When the disease is diagnosed early, before it is widespread and severe, it is easier to control and results in fewer complications. Unfortunately, early diagnosis is not typical. A person with pemphigoid may present with a single blister that does not heal. They may see several physicians and take several medications that do not work before they get a definitive diagnosis and the right treatments to control the disease so the blisters heal and new ones do not form.
Diagnostic testing may include:
- Skin biopsy with immunofluorescence. The tissue is stained with a dye and is examined under a special microscope to look for the presence of autoantibodies.
- Blood work to test for the autoantibodies that occur in pemphigoid.
Treatment of Pemphigoid
Treatment of pemphigoid may include steroids, immunosuppressants, immune globulin therapy and/or rituximab (e.g., Rituxan®). Immune globulin therapy is administered into the blood stream intravenously (IVIG). Steroids are typically initiated in high doses to get the disease under control and then are weaned. If during the weaning process blisters reappear and/or worsen, immunosuppressants are started and weaning is reattempted. If this is unsuccessful in controlling the blisters, IVIG and/or rituximab may be added to the treatment plan. In very severe cases, IVIG may be the first treatment prescribed.
Resources:
- The International Pemphigus and Pemphigoid Foundation
www.pemphigus.org - Information from the National Institutes of Health:
Questions and Answers about Pemphigus, Pemphigus information
References:
The information provided on this website is not medical advice, nor is it intended to be a substitute for medical advice, diagnosis, and treatment. Always seek the advice of a physician or other qualified health provider with questions concerning a medical condition. Never disregard professional medical advice, or delay seeking it based on information provided on this website.
