Stevens Johnson syndrome (SJS)

Last Updated on by FRCEM Intermediate

Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a very rare, acute, serious, and potentially fatal skin reaction, usually precipitated by medication, in which there is sheet-like skin and mucosal loss. It can affect all age groups and is slightly more common in females than males.

Commonly implicated drugs include:

  • Sulphonamides: co-trimoxazole
  • Beta-lactams: penicillins, cephalosporins
  • Anticonvulsants: lamotrigine, carbamazepine, phenytoin, phenobarbitone
  • Allopurinol
  • Paracetamol
  • Nevirapine (non-nucleoside reverse-transcriptase inhibitor)
  • NSAIDs (oxicam type mainly)

For most drugs, the onset is within a few days up to 1 month. There is usually a prodromal flu-like illness of several days duration before development of an acute onset tender, painful red rash starting on the trunk and extending rapidly over hours to days onto the face and limbs. The maximum extent is usually reached by 4 days. The appearance of the skin lesions may vary and may include: macules, purpuric spots, targetoid lesions (as in erythema multiforme), diffuse erythema, and flaccid blisters. The blisters then merge to form sheets of skin detachment, exposing red, oozing dermis

Mucosal involvement is prominent and severe, with at least 2 mucosal surfaces affected including:

  • Eyes (conjunctivitis, less often corneal ulceration, anterior uveitis, panophthalmitis) – red, sore, sticky, photosensitive eyes
  • Lips/mouth (cheilitis, stomatitis) – red crusted lips, painful mouth ulcers
  • Pharynx, oesophagus – causing dif􀃗culty eating
  • Genital area and urinary tract – erosions, ulcers, urinary retention
  • Upper respiratory tract (trachea and bronchi) – cough and respiratory distress
  • Gastrointestinal tract – diarrhoea

The patient is very ill and in considerable pain. In addition to skin/mucosal involvement, other organs may be affected including liver, kidneys, lungs, bone marrow and joints. SJS/TEN can be fatal due to complications in the acute phase. The mortality rate is up to 10% for SJS and at least 30% for TEN.

During the acute phase, potentially fatal complications include:

  • Dehydration and acute malnutrition
  • Infection of skin, mucous membranes, pneumonia, septicaemia
  • Acute respiratory distress syndrome
  • Gastrointestinal ulceration, perforation and intussusception
  • Shock and multiple organ failure including kidney failure
  • Thromboembolism and disseminated intravascular coagulopathy

SJS/TEN is suspected clinically and classified based on the skin surface area detached at maximum extent. Skin biopsy is usually required to con􀃗rm the clinical diagnosis and to exclude Staphylococcal Scalded Skin Syndrome (SSSS) and other generalised rashes with blisters.

Treatment of a patient with SJS/TEN involves:

  • Cessation of suspected causative drugs
  • Hospital admission – ITU or burns unit
  • Nutritional and fluid replacement (managed like a burn)
  • Temperature maintenance
  • Respiratory support – may require intubation and mechanical ventilation
  • Pain relief
  • Sterile handling and reverse isolation procedures
  • Skin care – monitor for secondary infection, topical antiseptics, medicated dressings
  • Eye care – review by ophthalmology, eye drops as indicated
  • Mouth care – mouthwashes, topical oral anaesthetic
  • Genital care if ulcerated – catheter
  • Physiotherapy to maintain joint movement
  • Assessment for secondary infection
  • Thromboprophylaxis

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