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Therapeutic Strategies

Chronic Bullous Disease of Childhood

Timothy Berger Bruce Wintroub

Thursday, July 15, 2010


Chronic bullous disease of childhood is characterized by the presence of multiple, tense bullae and severe itching in association with linear deposition of IgA at the basement membrane zone. The clinical appearance resembles bullous pemphigoid. It often remits 2-3 years after onset and rarely lasts past 10 years of age. Because this disorder affects children and is most frequently self-limited, the therapeutic strategy is to use minimally toxic agents that are likely to control the inflammatory process.

First Steps

  1. Treat with either dapsone 2 mg/kg/day or sulfapyridine 35-70 mg/kg/day.
  2. If no improvement is noted after 5 days, increase the dosage of either drug at 5-day intervals until an efficacious dose is reached. Sulfapyridine 200 mg to 2 g/day or dapsone 25-200 mg/day may be required. Either drug may be crushed in a flavored syrup. Monitor CBC prior to each increase in sulfone dose.

Subsequent Steps

When the disease is controlled, taper either drug to the dose required to maintain patient comfort. Attempt to wean the patient off medication at 4- or 6-month intervals. Recheck the CBC every 2 months while the patient is receiving maintenance sulfones.

Alternative Steps

  1. Although most children respond to sulfones, addition of corticosteroids (eg, prednisone) may be required to achieve control.
  2. If control is not possible with 2 g of sulfapyridine or 200 mg of dapsone, add prednisone 1 mg/kg/day and attempt to convert to a alternate day steroid schedule as quickly as possible.


  1. Because this is a benign, self-limited disorder, patient comfort rather than total suppression of disease activity is the therapeutic goal. Accept limited disease activity, and do not overtreat with steroids or sulfones in relatively recalcitrant cases.
  2. Screen all patients for G6PD activity prior to beginning sulfone therapy.

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