Stevens-Johnson Syndrome (SJS)/ Toxic Epidermal Necrosis (TEN)
Description Diagnostic criteria General and supportive measures Medicine treatment Referral ICD-10: L51.1 / L51.2 DESCRIPTION Life-threatening, acute hypersensitivity reaction with systemic upset, epidermal necrosis, and mucous membrane involvement. TEN and SJS are different ends of the same spectrum: in TEN, epidermal necrosis involves > 30% of body surface area, while in SJS the i
General and supportive measures
ICD-10: L51.1 / L51.2
DESCRIPTION
Life-threatening, acute hypersensitivity reaction with systemic upset, epidermal necrosis, and mucous membrane involvement. TEN and SJS are different ends of the same spectrum: in TEN, epidermal necrosis involves > 30% of body surface area, while in SJS the involvement is < 10%.
This condition is usually due to medication, e.g. sulphonamides, non-nucleoside reverse transcriptase inhibitors (especially nevirapine), antiepileptics (phenytoin, phenobarbitone, carbamazepine, lamotrigine), allopurinol, laxatives (phenolphthalein).
Complications include:
- dehydration, electrolyte disturbances and shock,
- hypoalbuminaemia,
- hypo- and more commonly hyperthermia,
- high output cardiac failure,
- secondary infection and sepsis; and
- adhesions and scarring.
DIAGNOSTIC CRITERIA
Cutaneous lesions may start as a dusky red macular rash, progressing to confluence with epidermal necrosis and large flaccid blisters which rupture, leaving large areas of denuded skin. Mucous membrane erosions are common and multi-organ involvement may be present.
GENERAL AND SUPPORTIVE MEASURES
- May require care in high or intensive care unit.
- Examine daily for systemic involvement, infection and ocular lesions. If infection is suspected, send blood and skin lesion specimens for culture and sensitivity before initiating antibiotic therapy.
- Do not puncture bullae or vesicles.
- Cool compresses and wet dressings.
- Regular supervised oral, genital and eye care to prevent adhesions and scarring.
- Encourage oral fluids, to prevent adhesions.
- Maintain fluid balance. Beware of shock.
- Nasogastric feeds if unable to eat; IV alimentation if enteral feeds are not possible.
- Stop all potentially causative medicines
MEDICINE TREATMENT
For pain
These patients require effective pain control.
Change of dressing protocol: See Pain Control.
Dressings
Skin hygiene, daily cleansing and bland, non-adherent dressings as needed.
| Do not use silver sulfadiazine if Stevens-Johnson syndrome is thought to be due to cotrimoxazole or other sulphonamide. |
Empiric antibiotic therapy
For secondary infections:
Use IV antibiotics if the oral route cannot be used.
- Cloxacillin, IV, 50 mg/kg/dose 6 hourly.
OR
- Cephalexin, oral, 25 mg/kg/dose 6 hourly.
Where weight is unknown:
◦ Child < 2 years: 125 mg.
◦ Child 2–10 years: 250 mg.
◦ > 10 years: 500 mg.
OR
- Flucloxacillin, oral, 25 mg/kg/dose 6 hourly.
Penicillin allergy:
- Clindamycin, oral, 6 mg/kg/dose, 6 hourly.
Reconsider choice of antibiotic when the results of cultures become available or the child does not improve.
For oral lesions
- Chlorhexidine 0.2%, 15 mL as a mouthwash.
- Use as needed.
- Do not swallow.
Note: The use of systemic corticosteroids is not recommended.
REFERRAL
- Discuss with a specialist, if considering re-initiation of medicine treatment.
Standard Treatment Guidelines And Essential Medicines List For South Africa, Hospital Level, Paediatrics, 2023 Edition
Chapter 5: Dermatology, 5.2 Erythema And Desquamation, 5.2.2 Stevens-Johnson Syndrome (SJS)/ Toxic Epidermal Necrosis (TEN)