Pathogenesis and A Practical Guide to the Management of Steven-Johnson Syndrome & Toxic Epidermal Necrolysis

Abstract

Steven Johnson Syndrome (SJS) and Toxic Epidermal necrolysis are rare dermatological emergencies that are associated with a high degree of morbidity and mortality [1]. They are considered to be severe blistering conditions that portray an image of diffuse epidermal necrolysis in association with sloughing of the skin. SJS and TEN are effectively the same disease with the main difference being the percentage of body surface area involved. It is widely accepted that SJS belongs to the below 10% body surface area category and TEN to the more than 30% category. A Steven Johnson syndrome and Toxic Epidermal Necrolysis overlap exists when the body surface area affected is between 10 percent and 30 percent [2]. The causes of both conditions within this spectrum are most likely drug related, however certain infections can also trigger SJS/TEN but this is less likely. In a substantial number of cases the cause is idiopathic [3]. The main grading system is the SCORTEN criteria, which can also provide more information regarding prognosis and mortality [4]. Management of this condition is highly controversial with no clear guidelines but there is a wide agreement that patients should be managed in a burns unit with intravenous fluids and protection from secondary infections. Stopping all medications and investigating for the cause is important unless there is clear benefit from any single medication.