Bullous Pemphigoid

Bullous pemphigoid

Bullous pemphigoid (BP) is a chronic blistering skin disease which ranges from mildly itchy welts to severe blisters and infection. A small area of the body can be affected or it can be widespread. BP has been seen at all ages but the usually affected are the elderly individuals.^[1] The disease is characterized by the presence of immunoglobulin G (IgG) autoantibodies specific for the hemidesmosomal bullous pemphigoid antigens BP230 (BPAg1) and BP180 (BPAg2).^[2]

Epidemiology

Bullous pemphigoid (BP) is uncommon but cases have been reported throughout the world. In Europe, bullous pemphigoid was identified as the most common subepidermal autoimmune blistering disease. Most treated patients have bullous pemphigoid that remits within 1.5-5 years. In 10-25% of patients, mucosa is involved. Men and women are equally affected. The disease primarily affects individuals in the fifth through seventh decades of life, with an average age at onset of 65 years. A rising incidence of infant-onset bullous pemphigoid has been reported.^[2]

Causes

The cause is unknown. BP is thought to be related to immune system disorders, certain other diseases, or use of some medications.^[3] It has been postulated that IgG immunoglobulins (antibodies) and activated T lymphocytes (white blood cells) attack components of the basement membrane, specifically a protein called BP antigen BP180, or less commonly BP230. Researches show that bullous pemphigoid appears to be more prevalent in patients that have had neurological disease, particularly stroke, dementia and Parkinson disease.^[4]

Symptoms

Some people may present with no symptoms, others may have mild redness and irritation. In severe cases, multiple blisters, called bullae are found. ^[3]  Bullae are large blisters (thin-walled sacs filled with clear fluid). Typically the skin in BP is very itchy and large, red welts and hives may emerge before or during the blister formation. The blisters are widespread and are usually found on the areas of the body that flex or move.^[1] Other symptoms include itching, rashes, mouth sores and bleeding gums.^[3]

Diagnosis

The history of the patient is obtained and physical examination is done with careful assessment of the skin. Confirmation of diagnosis is often made by a skin biopsy of the blister.^[4]

Treatment

 In mild cases, an antibiotic, usually tetracycline, is given. Niacin may also be prescribed along with the antibiotic. Most patients are treated with corticosteroids. To suppress the immune system, chemotherapy or medicines may be administered.^[3]

References:

1. http://www.aocd.org/skin/dermatologic_diseases/bullous_pemphigoid.html
2. http://emedicine.medscape.com/article/1062391-overview
3. http://www.nlm.nih.gov/medlineplus/ency/article/000883.htm
4. http://dermnetnz.org/immune/pemphigoid.html

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