With respect to Toxic Epidermal Necrolysis (TENS):
a) List the main causes. (20% marks)
b) Outline the management. (80% marks)
Viral e.g. Influenza, Coxsackie, Mumps
Bacterial e.g. GAS, Diphtheria, Mycoplasma
Multi-disciplinary approach with dermatology, plastics, ophthalmology. Best managed in specialised burns unit
Stop precipitating agents e.g. NSAID / allopurinol General Haemodynamic and respiratory support.
Reverse-Isolation in single room with room temperature increased to 30-320C.
Awareness of potentially high fluid loss: may require aggressive replacement
Wound care: Cover the denuded skin with anti-septic soaked dressings, vigilance for secondary skin infections. No role for prophylactic antibiotics.
Analgesia for painful skin lesions and for dressing change.
Eye care: look for conjunctival hyperemia, epithelial defect & pseudomembrane formation. Treat with topical lubricants, topical steroids and topical antibiotic, as guided by ophthalmology. Attempt to place lines through normal skin if possible
Cyclosporin: Early administration at the dose of 3-5mg/kg is beneficial and is recommended.
Steroids: The use of systemic corticosteroids has not been evaluated in clinical trials & remains controversial. Early observational studies indicated higher frequency of complications & death; but recent meta-analysis found that steroid treatment was associated with reduced risk of death. The dose, route, duration & timing of steroids remain uncertain.
Plasmapheresis: Reported to be beneficial in small series and case reports, but role still not well defined.
Anti-TNFα monoclonal antibodies e.g. infliximab has been used successfully in small series of patients, but not recommended.
It is remarkable that this SAQ on a relatively rare condition had a 58% pass rate. The last time toxic epidermal necrolysis came up (in Question 10 from the first paper of 2005), only 13% of the candidates passed. Presumably, of that 34-candidate cohort from 2005 (of whom 19 were successful), thirteen years later some proportion were senior college fellows and Part II examiners, and now they think this question is a really good idea from an assessment value standpoint.
The best literature for the management of TEN is unfortunately paywalled (Fromowitz et al, 2007). That particular article shines brightest because they incorporate a long (28-point) list of management recommendations from the University of Florida protocol. Fortunately, Schneider & Cohen have an even better article, which is more recent (2017). Additionally, the 2016 UK guidelines are available as a free PDF (Creamer et al, 2016). These and other resources have been remixed and recut into the summary below.
Shiga, Sarah, and Rob Cartotto. "What are the fluid requirements in toxic epidermal necrolysis?." Journal of Burn Care & Research 31.1 (2010): 100-104.
Fromowitz, Jeffrey S., Francisco A. Ramos‐Caro, and Franklin P. Flowers. "Practical guidelines for the management of toxic epidermal necrolysis and Stevens–Johnson syndrome." International journal of dermatology 46.10 (2007): 1092-1094.
Arévalo, José M., et al. "Treatment of toxic epidermal necrolysis with cyclosporin A." Journal of Trauma and Acute Care Surgery 48.3 (2000): 473-478.
Schneck, Jürgen, et al. "Effects of treatments on the mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis: a retrospective study on patients included in the prospective EuroSCAR Study." Journal of the American Academy of Dermatology 58.1 (2008): 33-40.
Barron, Stacy J., Michael T. Del Vecchio, and Stephen C. Aronoff. "Intravenous immunoglobulin in the treatment of S tevens–J ohnson syndrome and toxic epidermal necrolysis: a meta‐analysis with meta‐regression of observational studies." International journal of dermatology 54.1 (2015): 108-115.
Schneider, Jeremy A., and Philip R. Cohen. "Stevens-Johnson syndrome and toxic epidermal necrolysis: a concise review with a comprehensive summary of therapeutic interventions emphasizing supportive measures." Advances in Therapy34.6 (2017): 1235-1244.
Han, Feng, et al. "Successful treatment of toxic epidermal necrolysis using plasmapheresis: A prospective observational study." Journal of critical care 42 (2017): 65-68.
Paquet, Philippe, et al. "Effect of N-acetylcysteine combined with infliximab on toxic epidermal necrolysis. A proof-of-concept study." Burns 40.8 (2014): 1707-1712.
Hunger, Robert E., et al. "Rapid resolution of toxic epidermal necrolysis with anti-TNF-α treatment." Journal of allergy and clinical immunology 116.4 (2005): 923-924.
Wolkenstein, Pierre, et al. "Randomised comparison of thalidomide versus placebo in toxic epidermal necrolysis." The Lancet 352.9140 (1998): 1586-1589.
Creamer, D., et al. "UK guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis in adults 2016." British Journal of Dermatology 174.6 (2016): 1194-1227.