Toxic epidermal necrolysis
From Wikipedia, the free encyclopedia
ICD-10 | L51.2 |
---|---|
ICD-9 | 695.1 |
DiseasesDB | 4450 |
eMedicine | emerg/599 med/2291 |
MeSH | D004816 |
Toxic Epidermal Necrolysis (TEN) is a life-threatening, and usually drug-induced, dermatological condition. It is characterized by the detachment of the top layer of skin, (epidermis) from the lowers layer of the skin (dermis) all over the body.
There is broad agreement in the medical literature that TEN can be considered a more severe form of Stevens-Johnson syndrome and debate whether it falls on a spectrum of disease that includes erythema multiforme.[1][2]
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[edit] Pathogenesis
Microscopically, TEN causes cell death throughout the epidermis. Keratinocytes, which are the cells found lower in the dermis, specialize in holding the skin cells together, undergo necrosis (uncontrolled cell death).
[edit] Etiology
Toxic epidermal necrolysis is a rare and usually severe adverse reaction to certain drugs. The drugs most often implicated in TEN are certain antibiotics (sulfonamides (e.g. trimethoprim-sulfamethoxazole), penicillin, quinolones), NSAIDs (e.g. aspirin), acetaminophen, certain seizure drugs (carbamazepine, phenytoin, valproic acid), and corticosteroids.[citation needed]
[edit] Symptoms
TEN affects many parts of the body, but it most severely affects the mucous membranes, such as the mouth, eyes, and vagina. The severe findings of TEN are often preceded by 1 to 2 weeks of fever. These symptoms may mimic those of a common upper respiratory tract infection. When the rash appears it may be over large and varied parts of the body, and it is usually warm and appears red. In hours, the skin becomes painful and the epidermis can be easily peeled away from the underlying dermis. The mouth becomes blistered and eroded, making eating difficult and sometimes necessitating feeding through a nasogastric tube through the nose or a gastric tube directly into the stomach. The eyes are affected, becoming swollen, crusted, and ulcerated.
[edit] Diagnosis
Clinical, Histopathology[citation needed]
[edit] Treatment
First Line: early withdrawal of culprit drugs, early referral and management in burn units or intensive care units, supportive management, nutritional support
Second Line: Intravenous immunoglobulin (IVIG) - Uncontrolled trials showed promising effect of IVIG[citation needed] on treatment of TEN; a randomized control trial is needed in the future to determine the efficacy of IVIG in TEN.
Third Line: cyclosporin, cyclophosphamide, plasmapheresis, pentoxifylline, N-acetylcysteine, ulinastatin, infliximab, Granulocyte colony-stimulating factors (if TEN associated-leukopenia)
Systemic steroids are unlikely to offer any benefits.[citation needed]
[edit] Prognosis
The mortality for toxic epidermal necrolysis is 30-40 per cent.[citation needed] Deaths are caused either by infection or by respiratory distress which is either due to pneumonia or to the damage to airway linings.
[edit] References
- ^ Carrozzo M, Togliatto M, Gandolfo S (1999). "[Erythema multiforme. A heterogeneous pathologic phenotype]". Minerva Stomatol 48 (5): 217-26. PMID 10434539.
- ^ Farthing P, Bagan J, Scully C (2005). "Mucosal disease series. Number IV. Erythema multiforme". Oral Dis 11 (5): 261-7. PMID 16120111.
[edit] See also
[edit] External links
- Merck Home 18-203e
- Stevens Johnson Syndrome Foundation
- Stevens-Johnson syndrome Support
- Association of victims of medicines
- DermNetNZ
survivor