Title:Stevens-johnson Syndrome and Toxic Epidermal Necrolysis: An Overview
of Diagnosis, Therapy Options and Prognosis of Patients
Volume: 17
Issue: 2
Author(s): Vivek Kumar Garg*, Harpal Singh Buttar, Sajad Ahmad Bhat, Nuftieva Ainur, Tannu Priya, Dharambir Kashyap and Hardeep Singh Tuli
Affiliation:
- Department of Medical Lab Technology, Chandigarh University, Gharuan, Mohali, 140413, India
Keywords:
Stevens-johnson syndrome, toxic-epidermal necrolysis, skin and mucus membranes hypercreativity, severe adverse drug reactions, medication-related illnesses, cephalosporins.
Abstract: Both Stevens-johnson syndrome (SJS) and Toxic-epidermal necrolysis (TEN) are generally
medication-induced pathological conditions that mostly affect the epidermis and mucus
membranes. Nearly 1 to 2 patients per 1,000,000 population are affected annually with SJS and
TEN, and sometimes these maladies can cause serious life-threatening events. The reported death
rates for SJS range from 1 to 5%, and 25 to 35% for TEN. The mortality risk may even be higher
among elderly patients, especially in those who are affected by a significant amount of epidermal
detachment. More than 50% of TEN patients who survive the illness may experience long-term
lower quality of life and lesser life expectancy. The clinical and histopathological conditions of
SJS and TEN are characterized by mucocutaneous discomfort, haemorrhagic erosions, erythema,
and occasionally severe epidermal separation that can turn into ulcerative patches and dermal necrosis.
The relative difference between SJS and TEN is the degree of ulcerative skin detachment,
making them two extremes of a spectrum of severe cutaneous adverse drug-induced reactions
(cADRs). In the majority of cases, serious drug-related hypercreativities are considered the main
cause of SJS & TEN; however, herpes simplex virus and Mycoplasma pneumoniae infections may
also produce similar type clinical conditions. The aetiology of a lesser number of cases and their
underlying causative factors remain unknown. Among the drugs with a ‘greater likelihood’ of
causing TEN & SJS are carbamazepine (CBZ), trimethoprim-sulfamethoxazole, phenytoin, aminopenicillins,
allopurinol, cephalosporins, sulphonamides, antibiotics, quinolones, phenobarbital,
and NSAIDs of the oxicam variety. There is also a strong genetic link between the occurrence of
SJS and IEN in the Han Chinese population. Such genetic association is based on the human leukocyte
antigen (HLA-B*1502) and the co-administration of carbamazepine. The diagnosis of SJS
is made mostly on the gross observations of clinical symptoms, and confirmed by the histopathological
examination of dermal biopsies of the patients. The differential diagnoses consist of the
exclusion of Pemphigus vulgaris, bullous pemphigoid, linear IgA dermatosis, paraneoplastic pemphigus,
disseminated fixed bullous drug eruption, acute generalized exanthematous pustulosis
(AGEP), and staphylococcal scalded skin syndrome (SSSS). The management of SJS & TEN is
rather difficult and complicated, and there is sometimes a high risk of mortality in seriously inflicted
patients. Urgent medical attention is needed for early diagnosis, estimation of the
SCORTEN prognosis, identification and discontinuation of the causative agent as well as highdose
injectable Ig therapeutic interventions along with specialized supportive care. Historical aspects,
aetiology, mechanisms, and incidences of SJS and TEN are discussed. An update on the
genetic occurrence of these medication-related hypersensitive ailments as well as different therapy
options and management of patients is also provided.
