Infliximab for the Treatment of Adult-Onset Pityriasis Rubra Pilaris

Abstract

A 77-year-old man presented in June 2002 with a 2-month history of a progressive eruption that initially involved his scalp and progressed to his face, neck, trunk, and extremities. This eruption was associated with chills. His medical history was remarkable for congestive heart failure, hypertension, a mitral valve repair, and gout. His medications included warfarin sodium, digoxin, losartan potassium, hydrochlorothiazide, and terazosin hydrochloride. He had also been taking prednisone and cephalexin prescribed by his primary care physician for his skin condition. Physical examination revealed erythematous, scaly plaques and papules on his scalp, face, neck, upper trunk, and proximal extremities (Figure 1). He had ectropion of both lower eyelids, keratoderma of the palms and soles, and significant pitting edema of the lower extremities. A biopsy specimen was obtained and revealed parakeratosis, moderate acanthosis, and a mild superficial perivascular lymphocytic infiltrate, consistent with pityriasis rubra pilaris (PRP). Initial therapy consisted of acitretin, 25 mg twice daily, topical 0.1% triamcinolone acetonide ointment, and oral antihistamines. Prednisone therapy was tapered, and cephalexin therapy was discontinued. The patient was admitted to the hospital for intensive therapy with twicedaily whirlpool baths and to monitor his hemodynamic status. Lower extremity edema improved with hospitalization. His skin eruption improved only slightly. Acitretin therapy was continued as an outpatient; however, the skin eruption failed to improve. After 4 months of acitretin monotherapy, cyclosporine A was added at a daily dose of 3 mg/kg in October 2002. After 2 months of the combination therapy of acitretin with cyclosporine A, the patient’s skin eruption worsened, with increased total body skin area involvement and increased pruritus.