Recurrent erythema multiforme: Clinical characteristics, etiologic associations, and treatment in a series of 48 patients at Mayo Clinic, 2000 to 2007

BackgroundRecurrent erythema multiforme (EM) is a condition of substantial morbidity. Our efforts toward the etiologic attribution and treatment of recurrent EM have been less fruitful than those previously described.ObjectiveWe sought to further characterize clinical characteristics, etiologic associations, and treatment of recurrent EM.MethodsWe conducted a retrospective review of patients with recurrent EM seen between 2000 and 2007.ResultsOf 48 patients (mean age at disease onset, 36.4 years), 28 (58%) were female (mean duration of recurrent EM, 6 years). Thirty (63%) patients had oral involvement. Herpes simplex virus caused recurrent EM in 11 (23%) patients, and the cause remained unknown in 28 (58%). In all, 37 (77%) patients received systemic corticosteroids, 33 (69%) received continuous antiviral treatment, and 23 (48%) used immunosuppressive or anti-inflammatory agents. Sixteen of 33 patients receiving continuous antiviral treatment had either partial or complete disease suppression. Patients had varied responses to immunosuppressants, with mycophenolate mofetil providing partial or complete response in 6 of 8 patients. Features of recalcitrant cases included clinicians' inability to identify a specific cause, lack of improvement with continuous antiviral therapy, severe oral involvement, extensive corticosteroid therapy, and immunosuppressive therapy (two or more agents).LimitationThis study is retrospective.ConclusionsMore than half of patients in this study did not have an identifiable cause for recurrent EM, and herpes simplex virus was found less frequently than reported in previous studies. Response to systemic treatments, including continuous antivirals and immunosuppressants, was varied and oftentimes suboptimal.