Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

OVERVIEW

  • = immune complex mediated hypersensitivity -> severe erythema multiforme.
  • separation of the epidermis from the dermis
  • most authors believe toxic epidermal necrolysis (TEN) and SJS are different ends of the same spectrum of disease
  • erythema multiforme major is considered a distinct disorder
  • serious systemic disorder (multisystem)
  • mortality proportional to BSA

Disease nomenclature is based on the extent of the skin lesions

  • < 10% BSA = SJS
  • 10-30% BSA = SJS/ TEN overlap syndrome
  • > 30%= TEN

CAUSES (MIDI)

  • malignancy related (carcinomas and lymphomas)
  • infectious (viral, bacterial, fungal, protozoal)
  • drug induced (penicillins, sulfa drugs, quinolones, cephalosporins, anticonvulsants, COX-2, immunosuppressants, allopurinol, corticosteroids)
  • idiopathic

HISTORY

  • prodrome: systemic symptoms (1-14 days)
  • co-morbidities: HIV and SLE
  • painful and burning rash with mucosal involvement

EXAMINATION

  • mucocutaneous lesions: papules/vesicles -> clusters, nonpruritic involving all mucous membranes (oropharynx, airway, urethra, cornea) -> rupture leaving denuded skin
  • can be target lesions
  • %BSA involved
  • fluid status
  • nutritional status

INVESTIGATIONS

  • standard investigations involved in resuscitation
  • skin biopsy: subepidermal bullae, epidermal necrosis, perivascular lymphocytic infiltration
  • skin and blood cultures
  • bronchoscopy – airway involvement
  • endoscopy – GI involvement

MANAGEMENT

  • determined by the severity of the syndrome

Resuscitate

  • A – may need to be intubated c/o mucosal involvement
  • B – protective lung ventilation (can develop pulmonary complications: secretions, sloughing of bronchial epithelim, BOOP)
  • C – fluid resuscitation similar to burn patient, large volumes proportional to BSA involved, will have a hyperdynamic circulation with vasodilatory shock (managed with careful fluids and inotropic support), monitor end-organ function -> urine output >1mL/kg/hr
  • D – multimodal analgesia required -> may have to intubated and ventilated for analgesia
  • E – keep warm and isolated if possible to decrease risk of superinfection, humified environment, warm OT

Specific treatment

  • stop offending agent
  • identify and treat underlying disease and secondary infection (antibiotics)
  • burns dressings
  • antibiotics for documented invasive superinfection
  • avoid antibiotics that may exacerbate conditions (silver sulphadizine -> sulpha based)
  • IgG and steroids – controversial (EuroSCAR study)
  • consider plasma exchange

General treatment

  • careful management of fluid-balance and electrolyte abnormalities required
  • nutrition
  • thromboprophylaxis

Disposition

  • management in a burns unit if large TBSA involvement
  • keep family informed
  • consult dermatology and plastic surgery early and involve burns nurse

References and Links

CCC 700 6

Critical Care

Compendium

Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also the Innovation Lead for the Australian Centre for Health Innovation at Alfred Health and Clinical Adjunct Associate Professor at Monash University. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.

After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.

He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE.  He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.

His one great achievement is being the father of two amazing children.

On Twitter, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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