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Bullous pemphigoid

Senile Dermatitis Herpetiformis; Pemphigoid, Benign Pemphigus, Parapemphigus, Old Age Pemphigus

Overview

Bullous pemphigoid is an acute or chronic autoimmune skin disease, involving the formation of large blisters, more appropriately known as bullae, at the space between the skin layers epidermis and dermis. It is classified as a type II hypersensitivity reaction, with the formation of anti-hemidesmosome antibodies. The blisters are usually located on the arms, legs, or middle of the body. In some people, the mouth or genitals are also affected. The blisters may break open and form ulcers or open sores. Bullous pemphigoid usually occurs in older persons and is rare in young people. Symptoms may come and go. In most patients, the condition goes away after several years. Bullous pemphigoid is an autoimmune disorder which occurs when the body's immune system attacks and destroys healthy body tissue by mistake. Treatment may include corticosteroids taken by mouth or applied to the skin. Medicines that suppress the immune system may also be prescribed. For some, antibiotics in the tetracycline family are useful.

Symptoms - Bullous pemphigoid

Clinically, the earliest lesions may appear urticarial (like hives). Tense bullae eventually erupt, most commonly at the inner thighs and upper arms, but the trunk and extremities are frequently both involved. Any part of the skin surface can be involved. Oral lesions are present in a minority of cases.The disease may be acute, but typically will wax and wane. Several other skin diseases may have similar symptoms. However, milia are more common with epidermolysis bullosa acquisita, because of the deeper antigenic targets. A more ring-like configuration, with a central depression or centrally collapsed bullae may indicate linear IgA disease. Nikolsky's sign is negative unlike pemphigus vulgaris where it is positive.

Causes - Bullous pemphigoid

In most cases of bullous pemphigoid, no clear precipitating factors are identified. Potential precipitating events that have been reported include exposure to ultraviolet light and radiation therapy. Onset of bullous pemphigoid has also been associated with certain drugs, including furosemide, and other nonsteroidal anti-inflammatory agents, captopril, penicillamine, and antibiotics.

Prevention - Bullous pemphigoid

Not supplied.

Diagnosis - Bullous pemphigoid

Diagnosis is based on two biopsies of the skin, one submitted for routine H&E staining and one for immunofluorescence studies.

Prognosis - Bullous pemphigoid

Bullous pemphigoid may be self-resolving in a period ranging from several months to many years even without treatment. Poor general health related to old age is associated with a poorer prognosis.

Treatment - Bullous pemphigoid

Treatments include class I topical steroids (clobetasol, halobetasol, etc.) which in some studies have proven to be equally effective as systemic, or pill, therapy and somewhat safer. However, in difficult-to-manage or widespread cases, systemic prednisone and powerful steroid-free immunosuppressant medications, such as methotrexate, azathioprine or mycophenolate mofetil, may be appropriate. Antibiotics such as tetracycline or erythromycin may also control the disease, particularly in patients who cannot use corticosteroids. The anti-CD20 monoclonal antibody rituximab has been found to be effective in treating some otherwise refractory cases of bullous pemphigoid.

IgA-mediated pemphigoid can often be difficult to treat even with usually effective medications such as rituximab.

Resources - Bullous pemphigoid

  • NIH
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