Treatment of toxic epidermal necrolysis with cyclosporin A

Background: Toxic epidermal necrolysis (TEN) is a severe skin disorder char acterized by separation of the dermal-epidermal-junction, as is observed in second-degree superficial burns. It has been proposed that immunosuppressi ve treatment may improve prognosis of patients with TEN, Methods: We report here a case series of patients with TEN treated with cyc losporin A (CSA) without other concomitant immunosuppressive agent, These p atients (n = 11) were consecutively admitted to our Intensive Care Burn Uni t because of severe TEN, involving a large body surface area (83 +/- 17% [m ean +/- SD], median, 90%; range, 35-96%) and were treated with CSA 3 mg/kg per day enterally every 12 hours. We compared the series of patients treate d,vith CSA with a historical series of patients admitted to our Intensive C are Burn Unit before CSA was introduced as part of the treatment protocol, These patients (n = 6) were treated with cyclophosphamide (150 mg i,v, ever y 12 hours) and different doses of corticosteroids (greater than or equal t o 1 mg/kg per day of 6-methyl-prednisolone). Both groups of patients were s imilar in regard to age, delay front onset of disease to Intensive Care Bur n Unit admission, and body surface area involved. Results: Time from the onset of skin signs to arrest of the disease progres sion (1.4 +/- 0.3 days, vs, 3.6 +/- 1.5 days) and to complete reepitheliali zation (12.0 +/- 3.6 days, vs. 17.6 +/- 3.1 days) was significantly shorter in patients treated with CSA compared with those treated with cyclophospha mide and corticosteroids (p = 0,0002, and p = 0,0058, respectively). Signif icantly fewer patients in the CSA group had greater than or equal to 4 orga ns failing (2 of 11 vs. 3 of 6, respectively, p = 0,029), had severe leukop enia (<1000 cells/mu L) (0 of II vs. 4 of 6, respectively, p = 0,006), or d ied (3 of 6 vs. 0 of 11, respectively, p = 0,0029), Conclusion: We conclude that immunosuppressive treatment with CSA is safe a nd is associated with a rapid reepithelialization rate and a low mortality rate in patients with severe TEN, Our data suggest that this regimen could be more effective than treatment with cyclophosphamide and corticosteroids. Prospective controlled trials are required to test the hypothesis that CSA is more effective than cyclophosphamide or other immunosuppressive regimen s for the treatment of TEN.