By Disease Name > Stevens-Johnson Syndrome

Stevens-Johnson Syndrome

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extensive detachment of full thickness epidermis
no immunofluorescence at derm-epiderm jx (though may immune complexes in blood vessels of dermis)

 

prodrome:

usually begins as non-specific symptoms: fever, sore throat, burning eyes
1-3 days before cutaneous lesions

 

clinical:

a burning or painful rash starts symmetrically on the face and upper part of body and rapidly extends
poorly defined macules with dark purpuric centers, or atypical targets progressively merging
less frequently, the initial manifestation may be an extensive scarlatiniform erythema
mucous membrane involvement 85-95% (precedes skin rash in 1/3 of cases)

 

prognosis:

main cause of death = severe systemic infections
high fever is usual (a sudden drop in temperature is more indicative of sepsis)
usually erosions heal without scarring, but dyspigmentation is inevitable
post-TEN ocular syndrome

 

classic culprits:

almost always associated with drugs (sulfonamides most common)
sulfonamide antibiotics (e.g. Bactrim)
aromatic anticonvulsants (phenobarbital, phenytoin, carbamazepine)
other antibiotics (e.g. aminopenicillins)
some NSAIDS (e.g. phenylbutazone, oxyphenbutazone, isoxicam, piroxicam)
allopurinol
patients with HIV or SLE at increased risk

 

treatment:

do not use Silvadene (= silver sulfadiazine)
MONITOR FLUID AND ELECTROLYTES (with foley catheter for I&Os consider ICU)
warming
non-stick dressings;  change infrequently Q2- 3days (or when saturated with exudate)
ophtho consult to prevent ocular sequelae (e.g. synechiae)
ENT/nutrition consult catabolic state AND decreased PO intake secondary to sloughing mucous membranes of mouth/pharynx;  low threshold for NG tube
culture PRN early pathogen = staph aureus;  late = pseudomonas
consider transfer to Cedars at U of Miami (highest TEN survival rate in country)
do NOT treat with PO steroids; no evidence that it helps;  some evidence that it hurts
consider IVIg (at Cedars they give 1mg/kg/day X 4 days)
daily (or BID) cleansing with dilute chlorhexidine (hibiclens)
swab cultures from the skin daily for fungus and bacteria (dont treat the results, but let them guide your treatment IF the patient becomes ill systemically)
try to manage with NG tube and not an IV (entry portal for infection)