Recurrent erythema multiforme/Stevens-Johnson syndrome: response to mycophenolate mofetil.

A 26-year-old previously healthy man presented with a 1-month history of progressive mouth sores, eye discomfort, and skin rash. Over the 2 weeks before presentation, he had lost more than 10 kg (22 lb) because of difficulty in swallowing fluids and food, caused by the mouth sores. He had a history of herpes gladiatorum affecting his right shoulder. On physical examination, approximately 90% of his oral mucosa was ulcerated. Bilateral conjunctival hyperemia and targetoid skin macules involving his elbows, palms, upper thighs, and genitalia were observed (Figure 1). Results of 3 separate biopsies of the skin and mucosa were consistent with the clinical impression of erythema multiforme, showing scattered necrotic keratinocytes, basal cell liquefaction, subepidermal and intraepidermal edema, and a chronic inflammatory cell infiltrate (Figure 2). Three direct immunofluorescence studies showed intraepidermal cytoids and vascular staining, which were also consistent with erythema multiforme. The patient was admitted to the hospital for rehydration. He received oral corticosteroid therapy (prednisone, 1 mg/kg; 80 mg/d) and antiviral therapy (valacyclovir hydrochloride, 1 g twice daily). His condition improved. The prednisone was then decreased to 60 mg/d, and he was discharged from the hospital. Two days later, his mucosal ulcers and skin lesions recurred. The dose of prednisone was again increased to 80 mg/d, with a rapid disappearance of his mucosal ulcers and skin lesions within 2 days. Over the following 10 months, each time the dose of oral prednisone was reduced to below 80 mg/d, his disabling mouth sores, eye discomfort, and targetoid skin lesions recurred. The recurrence took place despite continuous antiviral therapy over this period (valacyclovir hydrochloride, 1 g twice daily, taken orally). Repeated cultures and polymerase chain reaction studies of skin and mucosa (both scrub cultures and biopsy material from a total of 17 samples submitted over this time period) were negative for virus, specifically for herpes simplex virus. THERAPEUTIC CHALLENGE