• | 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) |
• | patients with HIV or SLE at increased risk |
treatment:
• | do not use Silvadene (= silver sulfadiazine) |
• | MONITOR FLUID AND ELECTROLYTES (with foley catheter for I&O’s – consider ICU) |
• | 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 (don’t 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) |
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