Toxic Epidermal Necrolysis: A Clinical and Therapeutic Review.

Abstract

Toxic Epidermal Necrolysis is a rare dermatological condition with high mortality and serious consequences on its survivors. Despite having been first described in 1956, its pathophysiology remains uncertain, mainly regarding its mechanisms, although it seems that certain apoptosis pathways are pivotal in starting keratinocytes' apoptosis and in activating T cells, especially those mediated by tumour necrosis factor, Fas-FasL and granulysin. In general, its aetiology and presentation are consensual, being defined as a generalized necrolysis of the epidermis that occurs as an uncontrolled immune response to a specific drug or one of its metabolites, highlighting cotrimoxazole and allopurinol as the most important. This necrolysis leads to a massive shedding of the epidermal layer of the skin, with stronger incidences in the torso, upper limbs and face. Its complications tend to be severe, noting that septic ones are responsible for over half of the disease's mortality. Nearly all survivors develop long-term sequelae, namely hypertrophic scarring and skin pigmentation anomalies. Regarding treatment, many different opinions arise, including contradictory ones, regarding more importantly immunomodulation therapies that have been the focus of several studies through the years. It is safe to state that supportive therapy is the only modality that has significantly strong evidence backing its efficacy in reducing mortality and improving prognosis, which have improved in the past years as general health care quality increased. In conclusion, it is imperative to say that more research is needed for new potential therapies with large study populations and more scientific rigor. Likewise, investigation towards its basic pathophysiology should also be promoted, mainly at a biomolecular level, allowing for an improved prevention of this illness.