Management of bullous pemphigoid

Abstract

Bullous pemphigoid, the most common autoimmune blistering disease of the skin in adults, is caused by autoantibodies against hemidesmosomal adhesion proteins (BP180/type XVII collagen and BP230), leading into subepidermal blistering. Therefore patients, mostly older than 70years, show tight bullae and erosions of the skin and rarely at mucous membranes. Usually the disease shows achronically relapsing course. Thus there is aneed for long-term topical corticosteroids and if necessary systemic immunosuppressives. Still there is no curative treatment available. In the context of along-term treatment, drug-specific side effects and also a patient's comorbidities have to be taken into account. The choice of treatment should be based on disease activity and the extent of the muco-cutaneous manifestations. Dependent on this, high-potent topical class IV corticosteroids are used because of fewer side effects compared to systemic steroids. In case of an intense disease extent or in refractory courses treatment with systemic corticosteroids is usually combined with potentially corticosteroid-sparing immunomodulants such as dapsone or doxycycline or adjuvant immunosuppressives such as azathioprine, mycophenoles or methotrexate. Medium- to long-term an attendant immunosuppressant should be applied to reduce the use of corticosteroids. The CD20-antibody rituximab and high-dose intravenous immunoglobulins are also supplemental off-label options in refractory cases.