Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute life-threatening skin diseases characterized by extensive epidermal sloughing at the dermoepidermal junction resulting from keratinocyte apoptosis. 1 Both TEN and SJS are rare, affecting approximately 1 or 2 persons per 1, 000,000 populations annually, and are considered medical emergencies, as they are potentially fatal. 2
The exact molecular pathogenesis of TEN and SJS are not fully understood. TEN patients appear to, have an increased incidence of the haplotype HLA-B12, demonstrating an inability to detoxify intermediate reactive drug metabolites. 3 SJS and TEN are characterized by massive keratinocytes apoptosis. Fas-Fas ligand (FasL)-induced apoptosis in keratinocytes is one of the most thoroughly studied immune mechanisms in SJS/TEN. 4
At least 80% of TEN cases are believed to be …show more content…
ESR (first and second hours) was elevated in all patients. White blood cells and platelet counts were normal in all patients. Liver and renal function tests at the time of admission were normal in all patients. However, during hospital stay, liver function abnormality was observed in about 38% of the patients (8/21).
All patients received systemic steroids at a dose of 0.5mg/kg and antibiotics (macrolides).
The mean hospitalization duration for the 21 patients with SJS and TEN was 19.6±4.5 days (range, 15-30 days). The durations were 17.8 ±2.7 days (range, 15-24 days), 18. 37±2.9 (15-24 days) and 26.3±4. 8 (20-30 days) in the patients with SJS (n=14), SJS/TEN overlap (n=3) and TEN (n=4), respectively.
SCORTEN score 0 or- 1, 2, and 3 were found in 17 (81%), 3 (14.3%) and 1 (4.8%) of the patients (Table 4).
As regard to systemic complications, respiratory tract infections were found in 2 patients (9.5%), one with SJS/ TEN and the other with