Toxic epidermal necrolysis

Toxic epidermal necrolysis

Name = Toxic epidermal necrolysis

Caption =
DiseasesDB = 4450
ICD10 = ICD10|L|51|2|l|50
ICD9 = ICD9|695.1
MedlinePlus =
eMedicineSubj = emerg
eMedicineTopic = 599
eMedicine_mult = eMedicine2|med|2291
MeshID = D004816

Toxic epidermal necrolysis (TEN), also known as Lyell's syndrome, 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 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. [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] [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 |doi=10.1111/j.1601-0825.2005.01141.x] Some authors consider that there is an overlap between the two syndromes (usually between 10% and 30% of skin detachment).

The incidence is between 0.4 and 1.2 cases per million each year.


Microscopically, TEN causes cell death throughout the epidermis. Keratinocytes, which are the cells found lower in the epidermis, specialize in holding the skin cells together, undergo necrosis (cell death).


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; and 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 the herpes virus; and transplants of bone marrow or organs.


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. 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. [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 |doi=10.1016/j.jaad.2006.04.048]


The first line of treatment is early withdrawal of culprit drugs, early referral and management in burn units or intensive care units, supportive management, and nutritional support.

The second line is Intravenous immunoglobulin (IVIG) - Uncontrolled trials showed promising effect of IVIGFact|date=March 2007 on treatment of TEN; a randomized control trial is needed in the future to determine the efficacy of IVIG in TEN.

The third line is cyclosporin, cyclophosphamide, plasmapheresis, pentoxifylline, N-acetylcysteine, ulinastatin, infliximab, and/or Granulocyte colony-stimulating factors (if TEN associated-leukopenia exists).

Systemic steroids are unlikely to offer any benefits.Fact|date=March 2007


The mortality for toxic epidermal necrolysis is 30-40 per cent.Garra, GP (2007). " [ Toxic Epidermal Necrolysis] ". Retrieved on December 13, 2007.] 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. 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. [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 |doi=10.1001/archderm.141.6.683]


ee also

* Stevens-Johnson syndrome

External links

* [ Stevens Johnson Syndrome Foundation]
* [ DermNetNZ]

Wikimedia Foundation. 2010.

Игры ⚽ Поможем сделать НИР

Look at other dictionaries:

  • toxic epidermal necrolysis — n a skin disorder characterized by widespread erythema and the formation of flaccid bullae and later by skin that is scalded in appearance and separates from the body in large sheets called also epidermal necrolysis, Lyell s syndrome, scalded… …   Medical dictionary

  • epidermal necrolysis — n TOXIC EPIDERMAL NECROLYSIS …   Medical dictionary

  • necrolysis — Necrosis and loosening of tissue. [necro + G. lysis, loosening] toxic epidermal n. (TEN) a syndrome in which a large portion of the skin becomes intensely erythematous with epidermal necrosis, and peels off in the manner of a second degree burn,… …   Medical dictionary

  • Lyell syndrome — toxic epidermal necrolysis …   Medical dictionary

  • nonstaphylococcal scalded skin syndrome — toxic epidermal necrolysis …   Medical dictionary

  • TENS — toxic epidermal necrolysis syndrome; transcutaneous electrical nerve stimulation * * * TENS abbr transcutaneous electrical nerve stimulation; transcutaneous electrical nerve stimulator * * * see transcutaneous electrical nerve stimulation * * *… …   Medical dictionary

  • TENS — • toxic epidermal necrolysis syndrome; • transcutaneous electrical nerve stimulation …   Dictionary of medical acronyms & abbreviations

  • Stevens-Johnson syndrome — Infobox Disease Name = Stevens Johnson syndrome Caption = DiseasesDB = 4450 ICD10 = ICD10|L|51|1|l|50 ICD9 = ICD9|695.1 ICDO = OMIM = MedlinePlus = 000851 eMedicineSubj = emerg eMedicineTopic = 555 eMedicine mult = eMedicine2|derm|405 MeshID =… …   Wikipedia

  • Dermatitis exfoliativa neonatorum — Klassifikation nach ICD 10 L00 Staphylococcal scalded skin syndrome (SSS Syndrom) Dermatitis exfoliativa neonatorum (Ritter von Rittershain) Pemphigus acutus neonatorum Staphylogenes Lyell Syndrom …   Deutsch Wikipedia

  • Epidermolysis acuta toxica — Klassifikation nach ICD 10 L00 Staphylococcal scalded skin syndrome (SSS Syndrom) Dermatitis exfoliativa neonatorum (Ritter von Rittershain) Pemphigus acutus neonatorum Staphylogenes Lyell Syndrom …   Deutsch Wikipedia

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”