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Dr. Hibberd  

Treating Bullous Pemphigoid

Tuesday, August 30, 2011 8:22 AM

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Question: Do you know of any treatment or medication for the rare disease of bullous pemphigoid? I have had it for four years and now my gums are bleeding.

Dr. Hibberd's Answer:

Bullous pemphigoid is a rare immune mediated disorder where your body has produced antibodies to the basement membrane of your skin. This basement layer of cells connects the outer skin (epidermis) with the inner layer of skin (dermis) containing the nerves and capillaries needed to supply blood and deliver nutrients. The loss of basement membrane by attack from the immune system results in the formation of blisters with itching and hives.

Bullous pemphigoid seems to occur mainly in those over 60 years of age, and few triggers are recognized. Known triggers include radiation therapy, ultraviolet light therapy, and some medications used to treat fluid retention or rheumatoid arthritis.

Consultation with a dermatologist is advisable. You may be well-advised to see your periodontist also before events get more serious. You sound like you need more aggressive management by your description.

Bullous pemphigoid is usually treated with oral and/or injectable corticosteroids to suppress the immune system and improve symptoms. Topical corticosteroids may be useful for very early mild cases that don't involve the mouth or oral cavity. Treatment will often continue for several years into remission, and recurrences may occur when treatment stops. Sometimes chemotherapy and other medications are required to suppress the immune system. Antibiotics such as tetracycline with B vitamin (niacin) supplementation are often used in mild cases.

Complications may arise from infections at lesion sites, especially in the elderly with other medical conditions, and can become rapidly serious especially when the oral cavity becomes involved and nutrition and oral hygiene become impaired and difficult. Serious life-threatening complications are rare, but may occur especially in older adults or those with immune compromise. In those cases, infection may spread into the tissues and blood stream, and hospitalization, intravenous antibiotics, and aggressive disease suppression are required as well as multispecialty care.

Bullous pemphigoid must be differentiated from its bad-disease brother pemphigus (a potentially life-threatening disorder) by skin biopsy and evaluation by a skilled dermatopathologist as soon as the diagnosis is considered.

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