Steven Johnson's Syndrome

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CLINICAL DESCRIPTION

A hypersensitivity reaction to medications leading to necrosis and lysis of the epidermis affecting. Steven Johnson Syndrome (SJS) is in a spectrum with Toxic Epidermal Necrolysis (TEN) at the extreme end as follows: 

  • SJS: 1 to 10 % body surface area (BSA) involvement.
  • TEN/SJS overlap: between 10 and 30 % BSA
  • TEN: over 30 % BSA

CLINICAL FEATURES

SIGNS AND SYMPTOMS
  • Fever
  • Stinging, red eyes
  • Pain on swallowing
  • Pain and burning sensation on affected skin
  • Skin lesions tend to appear first on the trunk then spreading to the neck, face and proximal upper extremities
  • In the early stage, the skin may have a dusky brown appearance and later may form epidermal (flaccid) blisters which later break into painful erosions in areas of friction and of pressure
  • The affected skin is easily shorn off with minimal horizontal pressure (Nikolsky’s sign)
  • Mucous membrane involvement: red eyes, painful swallowing, dysuria, cough, erosions.

TREATMENT

  • Stop offending drugs immediately
  • Aim of treatment is to stop disease progression and to relieve symptoms.
  • Patients this condition must be managed in the hospital even if looking stable on first contact as the subsequent disease is unpredictable.
  • Appropriate analgesics
  • Fluid replacement
  • Give Ceftriaxone 2g IV daily for 5 days to minimize risk of infection
  • Short course steroids 1-2mg per kg daily for a maximum of 5 days
  • Refer for management at the hospital

Adjuvant Care

  • High protein diet is recommended
  • Ophthalmology management
  • Counselling to never take offending drug again