Toxic epidermal necrolysis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Toxic Epidermal Necrolysis is a life-threatening dermatological condition that is frequently induced by a reaction to medications. It is characterized by the detachment of the top layer of skin (the epidermis) from the lower layers of the skin (the 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]
Historical Perspective
Classification
Pathophysiology
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).
Causes
Toxic epidermal necrolysis is a rare and usually severe adverse reaction to certain drugs. History of medication use exists in over 95% of patients with TEN. The drugs most often implicated in TEN are antibiotics such as sulfonamides, nonsteroidal anti-inflammatory drugs, allopurinol, antiretroviral drugs, corticosteroids and anticonvulsants such as phenobarbital, phenytoin, carbamazepine, and valproic acid. The condition might also result from immunizations, infection with agents such as Mycoplasma pneumoniae or herpes virus and transplants of bone marrow or organs.
Differentiating Xyz from Other Diseases
Epidemiology and Demographics
The incidence is between 0.4 and 1.2 cases per 100,000 each year.
Risk Factors
Screening
Natural History, Complications, and Prognosis
The mortality for toxic epidermal necrolysis is 30-40%.[3] Loss of the skin leaves patients vulnerable to infections from fungi and bacteria, and can result in septicemia, the leading cause of death in the disease.[3] Death is caused either by infection or by respiratory distress which is either due to pneumonia or damage to the linings of the airway. Microscopic analysis of tissue (especially the degree of dermal mononuclear inflammation and the degree of inflammation in general) can play a role in determining the prognosis of individual cases.[4]
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Sometimes, however, examination of affected tissue under the microscope may be needed to distinguish it between other entities such as staphylococcal scalded skin syndrome. Typical histological criteria of TEN include mild infiltrate of lymphocytes which may obscure the dermoepidermal junction and prominent cell death with basal vacuolar change and individual cell necrosis.[5]
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Interventions
Surgery
Primary Prevention
Secondary Prevention
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.
- ↑ 3.0 3.1 Garra, GP (2007). "Toxic Epidermal Necrolysis". Emedicine.com. Retrieved on December 13, 2007.
- ↑ Quinn AM; et al. (2005). "Uncovering histological criteria with prognostic significance in toxic epidermal necrolysis". Arch Dermatol. 141 (6): 683–7. PMID 15967913.
- ↑ Pereira FA, Mudgil AV, Rosmarin DM (2007). "Toxic Epidermal Necrolysis". J Am Acad Dermatol. 56 (2): 181–200. PMID 17224365.