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Critical Situations
October 2000

Bullous Pemphigoid Treated With Leflunomide: A Novel Immunomodulatory Agent

Arch Dermatol. 2000;136(10):1204-1205. doi:10.1001/archderm.136.10.1204

A 70-year-old white woman presented with an 8-month history of worsening pruritus of the trunk and extremities, urticarial plaques on the trunk, and a few erythematous-based vesicobullous lesions on the lower extremities. She had a history of severe osteoporosis that was complicated by several vertebral fractures and incapacitating scoliosis. Her medications included pamidronate, calcitonin, calcium, and vitamin D. Histologic examination of the urticarial and bullous lesions showed a subepidermal blister with an intense eosinophilic infiltrate. Immunopathologic studies of the skin and serum samples confirmed the diagnosis of bullous pemphigoid. Prednisone therapy (30 mg/d [0.75 mg/kg per day]) was initiated. Complete resolution of the lesions and pruritus was achieved within 4 weeks, and the dosage of prednisone was tapered to 20 mg/d. A further tapering of the dosage of prednisone, to 15 mg/d, was associated with a significant flare of the disease. The dosage of prednisone was subsequently again increased to 20 mg/d, which was associated with a reinduction of complete remission. Several attempts at slowly tapering the prednisone dosage to less than 20 mg/d were unsuccessful in keeping the disease in remission. The patient refused to take steroid-sparing agents, eg, azathioprine, mycophenolate mofetil, and dapsone. Leflunomide therapy (20 mg/d) was initiated, and within 5 weeks, the dosage of prednisone was again slowly tapered to 15 mg/d. The prednisone therapy was continuously tapered and then discontinued 6 weeks later, and the dosage of leflunomide was decreased to 10 mg/d. Eight months after the patient began taking leflunomide, she remained in clinical remission on a regimen of 10 mg every other day. This regimen has been well tolerated, and the monthly complete blood cell counts and liver enzyme levels have remained normal.