Steroids used to desensitize penicillamine allergy in Wilson disease

Patients with Wilson disease require life-long treatment and penicillamine is the drug of choice. We present a 14-year-old boy with Wilson disease who developed hypersensitivity reaction 2 days after starting penicillamine therapy. His symptoms included fever, maculopapular rash and lip edema. The allergic reaction disappeared when penicillamine was discontinued, but relapsed after reinstituting penicillamine at a lower dose. Desensitization was attempted by introducing steroid therapy, the dose of 0.7 mg per kilogram per day of prednisolone for 2 days, then penicillamine was successfully tolerated. Subsequent tapering of the daily prednisolone dose was performed till it was discontinued one month after treatment began. We suggest that concomitant use of steroid and penicillamine should be used for the treatment of patients who develop penicillamine intolerance.