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Definition

Bullous Pemphigoid is a rare, autoimmune, chronic skin disorder. It is characterized by blistering. This disorder occurs mostly in elderly people. The disorder may occur in various forms, from no symptoms. It looks like mild redness and irritation, to multiple blisters. It is a chronic, autoimmune, subepidermal, blistering skin disease that rarely involves mucous membranes. Generalized blistering occurs in and under the upper layers of the skin and usually subsides spontaneously within several. In some rare cases of Bullous Pemphigoid, complications such as pneumonia may develop.

Causes

The exact cause of Bullous Pemphigoid is a autoimmune reaction by the body against the glue holding the skin together;  Autoimmune disorders are generated when the body’s natural defenses (e.g., the immune system with its antibodies) against “foreign” or invading organisms, attack healthy tissue for unknown reasons. It is caused by autoantibody mediated disruption of adhesion between basal keratocytes and the basement membrane, i.e., antibodies there binds the antigens of bullous pemphigoid. 

Bullous pemphigoid symptoms

Signs and symptoms of bullous pemphigoid range from mild to severe. Bullous pemphigoid most often shows itself in hard, tight blisters on the skin. The skin around the blisters may appear normal, but can be red and swollen or inflicted with a red scaly rash. Itching is common.

Treatment

Although bullous pemphigoid is a medical condition which can be irritating and troublesome, it is important to keep in mind that there are various ways in which individuals can attempt to control the occurrence of this rare condition.

Treatment often starts in hospital to ensure good control and so that proper dressings can be put on the blisters and raw areas.  Some patients can come off their treatment gradually, usually after a few months; others will need to go on with lower doses for a long time.  Calcium and vitamin D supplements, or other medications, should be given to keep your bones strong.

Treatment is focused on relief of symptoms and prevention of infection. Corticosteroids are the treatment of choice. They are given by mouth or by injection. Topical (applied to a localized area of the skin) corticosteroids may also be used on early, localized lesions.

Approximately 70% to 80% of patients with active BP have circulating antibodies to one or more basement membrane zone antigens. On direct immunofluorescence, the antibodies are deposited in a thin linear pattern; and on immune electron microscopy, they are present in the lamina lucida. Do not stop taking a corticosteroid drug suddenly. These types of drugs must be tapered off slowly under a doctor’s supervision.

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