Pathophysiology and management of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Abstract

Background:  Epidermal necrolysis (EN) is an acute mucocutaneous reaction syndrome characterized by extensive necrosis and epidermal exfoliation and can cause death. The initial lesion is in the form of erythematous macules, then progressively grow to sagging blister lesion and subsequent epidermal peeling. The causes of SJS and TEN may vary such as infections, vaccinations, drugs, systemic diseases, and food. However, the main cause is the drug as found in many cases.  Methods:  The pathophysiology of the EN is still unclear. Pathologically, tissue damage in the form of epidermal necrolysis is a picture of mass keratinocyte cell death through apoptosis. The stimuli that can induce apoptosis include cellular stress, DNA damage and intracellular cytokines. It may as a result of the role of cytotoxic T cells against keratinocytes, through perforin-granzyme B or Fas-FasL interactions and granulysin. Another theory of SJS-TEN pathophysiology is the slow acetylation (drug metabolic disorder) and the theory of genetic susceptibility. Result:  Finally, we include a comprehensive review of well-established widely available therapies in SJS-TEN patients.  Discussion:  It requires rapid diagnosis, suspension of suspected drug as soon as possible, supportive therapy and specific therapy Conclusion:  An understanding of the pathophysiology and current management of SJS and TEN is expected to assist in the prevention of disease and early diagnosis and can provide more effective therapy for SJS-TEN treatment   Keywords:  Epidermal necrolysis, SJS-TEN, perforin-granzyme B, Fas-FasL interactions, granulysin