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'''Toxic Epidermal Necrolysis''' is a life-threatening [[dermatology|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]].<ref>{{cite journal |author=Carrozzo M, Togliatto M, Gandolfo S |title=[Erythema multiforme. A heterogeneous pathologic phenotype] |journal=Minerva Stomatol |volume=48 |issue=5 |pages=217-26 |year=1999 |pmid=10434539}}</ref><ref>{{cite journal |author=Farthing P, Bagan J, Scully C |title=Mucosal disease series. Number IV. Erythema multiforme |journal=Oral Dis |volume=11 |issue=5 |pages=261-7 |year=2005 |pmid=16120111}}</ref> | '''Toxic Epidermal Necrolysis''' is a life-threatening [[dermatology|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]].<ref>{{cite journal |author=Carrozzo M, Togliatto M, Gandolfo S |title=[Erythema multiforme. A heterogeneous pathologic phenotype] |journal=Minerva Stomatol |volume=48 |issue=5 |pages=217-26 |year=1999 |pmid=10434539}}</ref><ref>{{cite journal |author=Farthing P, Bagan J, Scully C |title=Mucosal disease series. Number IV. Erythema multiforme |journal=Oral Dis |volume=11 |issue=5 |pages=261-7 |year=2005 |pmid=16120111}}</ref> | ||
==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 [[anticonvulsant]]s 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 [[Organ transplant|transplant]]s of [[bone marrow]] or organs. | |||
==Epidemiology and Demographics== | |||
The [[incidence (epidemiology)|incidence]] is between 0.4 and 1.2 cases per 100,000 each year. | |||
==Natural History, Complications and Prognosis== | |||
The mortality for toxic epidermal necrolysis is 30-40 per cent.<ref name=garra>Garra, GP (2007). "[http://www.emedicine.com/EMERG/topic599.htm Toxic Epidermal Necrolysis]". Emedicine.com. Retrieved on December 13, 2007.</ref> 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.<ref name=garra/> 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.<ref>{{cite journal |author=Quinn AM et al |title=Uncovering histological criteria with prognostic significance in toxic epidermal necrolysis |journal=Arch Dermatol |volume=141 |issue=6 |pages=683-7 |year=2005 |pmid=15967913}}</ref> | |||
==Diagnosis== | |||
===History and Symptoms=== | |||
TEN affects many parts of the body, but it most severely affects the [[mucous membrane]]s, 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. Often, the diagnosis can be made clinically. Generally, if the clinical history is consistent with Stevens-Johnson syndrome, and the skin lesion covers greater than 30% of the body surface area, the diagnosis of TEN is appropriate. Sometimes, however, examination of affected tissue under the microscope may be needed to distinguish it between other entities such as [[staphylococcal scalded skin syndrome]]. | |||
===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.<ref>{{cite journal |author=Pereira FA, Mudgil AV, Rosmarin DM |title=Toxic Epidermal Necrolysis |journal=J Am Acad Dermatol |volume=56 |issue=2 |pages=181-200 |year=2007 |pmid=17224365}}</ref> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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]
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 drugscorticosteroids 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.
Epidemiology and Demographics
The incidence is between 0.4 and 1.2 cases per 100,000 each year.
Natural History, Complications and Prognosis
The mortality for toxic epidermal necrolysis is 30-40 per cent.[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
History and 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. Often, the diagnosis can be made clinically. Generally, if the clinical history is consistent with Stevens-Johnson syndrome, and the skin lesion covers greater than 30% of the body surface area, the diagnosis of TEN is appropriate. Sometimes, however, examination of affected tissue under the microscope may be needed to distinguish it between other entities such as staphylococcal scalded skin syndrome.
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]
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.