Stevens-Johnson Syndrome (SJS)

Last updated 15.01.14

 

Definition

-  Very rare, potentially fatal, skin reaaction causing sheet like skin & mucosal loss typically caused by medication

 

Features

-  Dermatological emergency: esp elderly patients

- More commonly associated with HIV

- Onset after medications can take 1 day to 1 month 

 

Causes

-  Medication - associated with 200+

-  Antibiotics

- Typically within first week

- Sulphonamides (most common)

- Beta Lactams: Penicillins, Cephalosporins

- Antifungals: Imidazoles

- Antivirals: Nevirapira

- NSAIDs

- Anticonvulsants : typically within 2 months

- Vaccintaions (rarely)

- Mycoplasma infections

 

Associated S/S

-  Prodrome

- Fever

- Cough

- Sore throat, dysphagia

- Rhinitis

- Conjunctivitis

- Myalgia

- Rash

- Abrupt onset

- Usually starts on trunk/face & extends to limbs over hours to days

- Reaches maximum extent by 4 days

- Macules, targets, blisters & papules

- Blisters merge & skin sheds

- Nikolsky sign: blisters form when skin rubbed gently

- Beta Lactams: Penicillins, Cephalosporins

- Anxiety

- Pain

- 2+ mucosal surfaces effectes  

- Conjunctivitis

- Chelitis, stomatitis

- Respiratory: cough

- GIT: diarrhoea, oesophagitis

- Genital/rinary ulcers

 

SJS

 

Dx

-    Clinical Dx: <10% body surface skin detachment, if >10% = TEN 

 

SCORETEN

-  Predicts mortality

- 1 point for presence of each

- Age >40yrs

- Malignancy

- HR >120

- Urea >10mmol/L

- Glucose >14mmol/L

- Bicarb <20mmol/L

- >10% body surface effected

- Score 0-1: 3.2%

- Score 2: 12.1%

- Score 3 = 35.3%

- Score 4 = 38.3%

- Score 5+ >90%

   

Complications

-  Dehydration

- Malnutrition

- Infection: skin, pneumonia, sepsis

- Shock: multiple organ failure

- VTE: DVT, PE

- DIC

- Skin scarring

- Joint contractures

- Pigment changes

- Loss of nails

- Blindness

- Conjunctivities

- Corneal ulcers

- Symblepharon

- Ectropion

- Eutropion

- Trichiasis

- Synechiae

 

Ix

-  +/- Serum granulysin (in first few days)

- Skin Bx: exclude Staph Scolded Skin Syndrome

- Keratocyte necrosis

- Full thickness epidermal/epithelial necrosis

- Minimal inflammation

- FBE

- Anaemie: most patient

- Leukopaenia

- Neutropaenia: pooor prognosticator

- LFT: mildly deranged 30% pts

-  U&E

- Urine FWT: mild proteinuria 50%

- BSL

 

Mx

- Similar to burns

- Cease medication

- Referral to Dermatology

- Admit +/- ICU

- IV fluids +/- NGT

- Temperature maintenance

- Analgesia

- Sterile handling

- Skin Care

- Topical antiseptics

- Dressings

- Leave skin intact as biological dressing

- Eye Care

- Antiseptic drops

- Antibiotics drops

- Corticosteroids

- Mouth Care

- Mouth wash

- Anaesthetic

- +/- Intubation/ventilation

- IDC

- Physio

- Assess for infection regularly

- Tetanus

- Recovery over weeks to months

 

 

References

 

DermNet: SJS

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