Bullous Pemphigoid

Definition

Definition of Bullous Pemphigoid
Bullous pemphigoid, also referred to as BP, is an acute or chronic autoimmune skin disease, involving the formation of blisters, more appropriately known as bullae, at the space between the skin layers epidermis and dermis.

Symptoms

Symptoms of 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. Milia are more common with epidermolysis bullosa acquisita (EBA), because of the deeper antigenic targets. A more ring-like configuration, with a central depression or centrally collapsed bullae may indicate linear IgA disease. The disease may be acute, but typically will wax and wane.

Causes

Causes of Bullous Pemphigoid
The cause is not known, but may be related to immune system disorders, certain other diseases, or use of some medications.

Diagnosis

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

Treatment

Treatment of Bullous Pemphigoid
Treatments include Class I topical steroids (clobetasol, halobetasol, etc.) which in some studies have proven to be equally as 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.

Prognosis

Prognosis of Bullous Pemphigoid
Bullous pemphigoid usually responds well to treatment. Most patients may stop taking medicine after several years. However, the disease sometimes returns after treatment is stopped.

Prevention

Prevention of Bullous Pemphigoid
Consult with your doctor.