Saturday, January 01, 2011
Bullous pemphigoid is usually a self-limited inflammatory
subepidermal blistering disorder of the elderly, in which
circulating auto antibodies to epitopes in the basement membrane
zone bind to this region and initiate a cascade of inflammatory
events resulting in urticarial and bullous lesions. The therapeutic
strategy is to reduce auto antibody production and to control the
inflammatory reaction. Because bullous pemphigoid is not
life-threatening and agents that reduce auto antibody formation are
inherently high risk, the initial goal of therapy is to control
inflammation and reduce blister formation to a level consistent
with individual patient comfort.
- Treat with oral prednisone alone at 40-60 mg/ day in a single
morning dose. A dramatic clinical response will be seen in 70-80%
of patients after 2-3 weeks.
- Clobetasol ointment or another superpotent topical steroid is
applied to the affected areas twice daily.
Over 2-8 weeks, taper the prednisone dose to 30 mg/day by
reduction of the daily dose by 10 mg every 2-3 weeks. Then, over
the next 2-3 months, reduce prednisone to 10-20 mg daily. After
several months, shift the patient to alternate steroid therapy,
reaching 10 mg every other day. When 10 mg every other day is
reached, taper dose by 1 mg every other day every 1-4 weeks until
the lowest amount of prednisone controlling the appearance of new
lesions is reached.
Alternative agents may be used in patients who do not rapidly
respond to systemic steroids or in patients in whom the use of
systemic steroids is contraindicated.
- If the patient has complications or side effects from the
prednisone, has persistent severe disease, or if sufficient
clinical response is not achieved after 3 weeks, begin an
immunosuppressive agent in combination with prednisone. In each
case, continue the immunosuppressive agent for at least 2 months
before abandoning it as ineffective. Once the steroid-sparing agent
has begun to work, the dose of systemic steroids is gradually
tapered following the protocol above. The choice of
immunosuppressive agent is determined by the patient's coexistent
medical conditions, the risk of complications, and the cost. There
is no evidence that one agent is superior to another.
Steroid-sparing immunosuppressives include:
- Mycophenolate mofetil 500 mg - 1.5 mg twice daily (1.0-3.0 g
- Azathioprine 100-150 mg/day
- Methotrexate 5mg to 25 mg once weekly as a single oral
- Cyclophosphamide 50-150 mg/day
- Adjunctive measures that may be added to prednisone or
prednisone plus immunosuppressive agents.
- Tetracycline 500 mg three or four times daily plus niacinamide
500 mg three times daily. This may be adequate alone to control
mild disease or may provide additional antiinflammatory effect
without causing additional immunosuppression.
- Plasmapheresis to remove circulating pemphigoid autoantibody
may be of value, but should be combined with cyclophosphamide
50-150 mg to inhibit antibody production.
- Intravenous immunoglobin infusions (IVIG) may be used in
refractory cases. The dose is 2g/kg given over 3-5 days. This is
very costly and is less frequently required than in pemphigus
- Patients with bullous pemphigoid are often elderly and frail.
They tolerate high-dose systemic steroid therapy poorly. Do not be
reluctant to add low doses of a steroid-sparing agent to allow for
reduction in the systemic steroid dose. Complications usually occur
when systemic steroid dose is not reduced judiciously.
- Potential complications of systemic steroids and
immunosuppressants must be carefully sought in all patients so
- Other bullous diseases such as epidermolysis bullosa acquisita,
erythema multiforme, and bullous drug eruptions may clinically
resemble pemphigoid and may explain atypical therapeutic
- Immunosuppression may result in reactivation of herpes simplex
or varicella zoster virus infection. These bullous infectious
diseases may be generalized and simulate a flare of the