Treat with corticosteroids
The traditional treatment of BP is high-dose oral corticosteroids. However, long-term use of systemic corticosteroids can cause significant morbidity and has been linked to an increased mortality rate in the elderly population.4 A potent topical corticosteroid, such as clobetasol propionate cream 10 to 30 g/d tapering over 4 months, or 40 g/d tapering over 12 months,5,6 is an effective alternative (strength of recommendation [SOR]: A).
Other options include methotrexate, mycophenolate, azathioprine, niacinamide, doxycycline, intravenous (IV) immunoglobulin, and plasma exchange. These therapies are typically used in combination with corticosteroids, or after initial treatment failure. Evidence regarding their effectiveness is limited7 (SOR: B).
Although the disease is occasionally self-limited after the initial episode, most patients with BP will achieve clinical remission with medical intervention. Patients often experience recurrent outbreaks and require chronic use of immunosuppressive agents.
Our patient required ongoing care
Our patient was prescribed prednisone 80 mg/d PO in combination with topical clobetasol cream. Despite these treatments, the disease progressed. One week later, approximately 80% of his body surface was involved. He was admitted for fluid replacement and monitoring for infection.
Subsequent initiation of methotrexate, niacinamide, doxycycline, and topical clobetasol led to clinical remission. Unfortunately, the patient relapsed approximately 3 months later and required a second hospital stay.
In the ensuing months, the patient’s course was marked by frequent relapses and significant morbidity. Further treatment trials have included IV immunoglobulin, mycophenolate, and azathioprine.
CORRESPONDENCE
Casey Z. MacVane, MD, MPH, Department of Emergency Medicine, Maine Medical Center, 47 Bramhall Street, Portland, ME 04102; macvac1@mmc.org