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Keywords:

  • cutaneous sarcoidosis;
  • granulomatous disease;
  • sarcoidosis;
  • tumour necrosis factor alpha antagonists

ABSTRACT

The mortality rate of Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is high; approximately 5% for SJS and 25% for TEN. It is therefore vital for the treating physician to recognise SJS and TEN promptly through the identification of these diseases' characteristic clinical features so that the offending drug is promptly withdrawn, supportive therapy is administered and adjunctive therapies are considered. Supportive therapy addressing the manifestations and complications of acute skin failure include monitoring the fluid – electrolyte balance and providing enteral or parenteral nutrition, wound care and treatment of sepsis. In addition, supportive care of the affected mucosal surfaces is required through the use of aggressive ocular lubrication, topical corticosteroids, hygienic mouthwashes and oral anaesthetics, together with monitoring for urinary retention. There is sufficient evidence for the use of intravenous immune globulin in the acute management of SJS/TEN provided an adequate dose of 2–3 g/kg is administered. Cyclosporine appears to also be an effective agent although randomised control studies are required to demonstrate its benefit and establish the dose, duration of therapy and safety profile. The role of corticosteroids is currently under revision. Some earlier studies have shown a lack of efficacy or increased mortality in their use but the use of high doses early in the course of disease may actually reduce morbidity and mortality. The role of plasmapheresis, anti-tumour necrosis factor (TNF) biologics and N-acetylcysteine is promising but further studies are required to elucidate their benefit. Preventative strategies such as pharmacogenetic screening needs to be strongly considered, with the provision of cost-effective assays with a rapid turn-around time.