Toxic epidermal necrolysis
From Ganfyd
Toxic epidermal necrolysis (TEN, toxic epidermal necrolysis syndrome, TENS) is a serious life-threatening immunological skin condition usually caused by an idiosyncratic reaction to a medicine.
Contents |
Introduction
There is no consensus on the separation from Stevens-Johnson syndrome and the two seem to share very similar mechanisms. There is marked exfoliation of the skin with generalised erythema. The historic association with sulphonamides and barbiturates is noted. Many drugs are no longer used commonly or have had their license removed when others with similar indications have proved not to have this side-effect. Presently the drugs most likely to cause the conditions seem to be[1]:
- Allopurinol
- Carbamazepine
- Phenytoin
- Phenobarbitone
- Valproic acid
- Oxicam (NSAIDs)
- Corticosteroids (association is complicated)
 | Admit - A dermatology/burns emergency |
Aetiology
Activation of CD8 lymphocytes plays a role. There are HLA and racial associations but these appear to be drug specific.
Prognosis
Worse with delay in hospitalization, thrombocytopenia, and early empiric antibiotic treatment[2]
| SCORTEN score[3] has been validated[4] |
- Related to degree of dermal mononuclear inflammation[5]
- Mortality rate of 20 to 60%[6]
- Mortality predicted by:
- Sepsis
- Co-morbidities
- Age > 60 years
- Body surface area involved > 60%
- Long term survivors may have problems with[7]:
- Eyes (54%)
- Skin (81% - not recurrent TEN)
- Renal failure (23%)
Treatment
As for severe burns:
- Maintain fluid balance
- Control of secondary infection
- Nutritional support.
No convincing evidence for immunosuppressive therapies[8] including steroids and arguably these should only be given in a clinical trial context[9].
- Withdraw the offending drug. The following have strong enough associations to be considered causal in the the EuroSCAR study[10]:
- Allopurinol
- Carbamazepine
- Lamotrigine
- NSAIDs - Acetic acid and oxicam derivatives
- Nevirapine
- Phenobarbital
- Phenytoin
- Sertraline
- Sulfamethoxazole and other sulfonamides
- Quinolones
- Cephalosporins
- Aminopenicillins
- Macrolides
There is a fairly good predictive algorithm called ALDEN for other drugs[10]
References
- ↑ Halevy S, Ghislain PD, Mockenhaupt M, Fagot JP, Bouwes Bavinck JN, Sidoroff A, Naldi L, Dunant A, Viboud C, Roujeau JC. Allopurinol is the most common cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. Journal of the American Academy of Dermatology. 2008 Jan; 58(1):25-32.(Link to article – subscription may be required.)
- ↑ Schulz JT, Sheridan RL, Ryan CM, MacKool B, Tompkins RG. A 10-year experience with toxic epidermal necrolysis. The Journal of burn care & rehabilitation. 2000 May-Jun; 21(3):199-204.
- ↑ Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. The Journal of investigative dermatology. 2000 Aug; 115(2):149-53.(Link to article – subscription may be required.)
- ↑ Cartotto R, Mayich M, Nickerson D, Gomez M. SCORTEN Accurately Predicts Mortality Among Toxic Epidermal Necrolysis Patients Treated in a Burn Center. Journal of burn care & research : official publication of the American Burn Association. 2008 Jan-Feb; 29(1):141-6.(Link to article – subscription may be required.)
- ↑ Quinn AM, Brown K, Bonish BK, Curry J, Gordon KB, Sinacore J, Gamelli R, Nickoloff BJ. Uncovering histologic criteria with prognostic significance in toxic epidermal necrolysis. Archives of dermatology. 2005 Jun; 141(6):683-7.(Link to article – subscription may be required.)
- ↑ Ducic I, Shalom A, Rising W, Nagamoto K, Munster AM. Outcome of patients with toxic epidermal necrolysis syndrome revisited. Plastic and reconstructive surgery. 2002 Sep 1; 110(3):768-73.
- ↑ Oplatek A, Brown K, Sen S, Halerz M, Supple K, Gamelli RL. Long-term follow-up of patients treated for toxic epidermal necrolysis. Journal of burn care & research : official publication of the American Burn Association. 2006 Jan-Feb; 27(1):26-33.(Link to article – subscription may be required.)
- ↑ Schneck J, Fagot JP, Sekula P, Sassolas B, Roujeau JC, Mockenhaupt M. Effects of treatments on the mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis: A retrospective study on patients included in the prospective EuroSCAR Study. Journal of the American Academy of Dermatology. 2008 Jan; 58(1):33-40.(Link to article – subscription may be required.)
- ↑ Brown KM, Silver GM, Halerz M, Walaszek P, Sandroni A, Gamelli RL. Toxic epidermal necrolysis: does immunoglobulin make a difference? The Journal of burn care & rehabilitation. 2004 Jan-Feb; 25(1):81-8.(Link to article – subscription may be required.)
- ↑ a b Sassolas B, Haddad C, Mockenhaupt M, Dunant A, Liss Y, Bork K, Haustein UF, Vieluf D, Roujeau JC, Le Louet H. ALDEN, an algorithm for assessment of drug causality in Stevens-Johnson Syndrome and toxic epidermal necrolysis: comparison with case-control analysis. Clinical pharmacology and therapeutics. 2010 Jul; 88(1):60-8.(Link to article – subscription may be required.)
