Stevens Johnson Syndrome
5/8/10
SP Notes
= immune complex mediated hypersensitivity -> severe erythema multiforme.
- separation of the epidermis from the dermis
- most authors believe toxic epidermal necolysis (TEN) and SJS are different manifestations of
the same disease.
- serious systemic disorder (multisystem)
- mortality proportional to BSA
- < 10% BSA -> SJS
- > 30% TEN
CAUSES (MIDI)
- malignancy related (carcinomas and lymphomas)
- infectious (viral, bacterial, fungal, protozoal)
- drug induced (penicillins, sulfa’s, quinolones, cephalosporins, anticonvulsants, COX-2,
immunosuppressants, allopurinol, corticosteroids)
- idiopathic
HISTORY
- prodrome: systemic symptoms (1-14 days)
- mucocutaneous lesions: papules/vesicles -> clusters, nonpruritic involving all mucous
membranes (oropharynx, airway, urethra, cornea) -> rupture leaving denuded skin
- can be target lesions
- painful and burning
- co-morbidities: HIV and SLE
EXAMINATION
Jeremy Fernando (2011)
INVESTIGATIONS
-
standard investigations involved in resuscitation
skin biopsy: subepidermal bullae, epidermal necrosis, perivascular lymphocytic infiltration
skin and blood cutures
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
Treatment
Specific
-
stop offending agent
identify and treat underlying disease and secondary infection (antibiotics)
burns dressings
early consultation with dermatologist
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
- 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
Jeremy Fernando (2011)