Stevens-Johnson syndrome/toxic epidermal necrolysis
Last updated 18 Aug 2020
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Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a severe skin reaction most often triggered by particular medications. Although Stevens-Johnson syndrome and toxic epidermal necrolysis were once thought to be separate conditions, they are now considered part of a continuum. Stevens-Johnson syndrome represents the less severe end of the disease spectrum, and toxic epidermal necrolysis represents the more severe end.
Description
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a severe skin reaction most often triggered by particular medications. Although Stevens-Johnson syndrome and toxic epidermal necrolysis were once thought to be separate conditions, they are now considered part of a continuum. Stevens-Johnson syndrome represents the less severe end of the disease spectrum, and toxic epidermal necrolysis represents the more severe end.
SJS/TEN often begins with a fever and flu-like symptoms. Within a few days, the skin begins to blister and peel, forming very painful raw areas called erosions that resemble a severe hot-water burn. The skin erosions usually start on the face and chest before spreading to other parts of the body. In most affected individuals, the condition also damages the mucous membranes, including the lining of the mouth and the airways, which can cause trouble with swallowing and breathing. The painful blistering can also affect the urinary tract and genitals. SJS/TEN often affects the eyes as well, causing irritation and redness of the conjunctiva, which are the mucous membranes that protect the white part of the eye and line the eyelids, and damage to the clear front covering of the eye (the cornea).
Severe damage to the skin and mucous membranes makes SJS/TEN a life-threatening disease. Because the skin normally acts as a protective barrier, extensive skin damage can lead to a dangerous loss of fluids and allow infections to develop. Serious complications can include pneumonia, overwhelming bacterial infections (sepsis), shock, multiple organ failure, and death. About 10 percent of people with Stevens-Johnson syndrome die from the disease, while the condition is fatal in up to 50 percent of those with toxic epidermal necrolysis.
Among people who survive, long-term effects of SJS/TEN can include changes in skin coloring (pigmentation), dryness of the skin and mucous membranes (xerosis), excess sweating (hyperhidrosis), hair loss (alopecia), and abnormal growth or loss of the fingernails and toenails. Other long-term problems can include impaired taste, difficulty urinating, and genital abnormalities. A small percentage of affected individuals develop chronic dryness or inflammation of the eyes, which can lead to increased sensitivity to light (photophobia) and vision impairment.
Inheritance patterns
The source lists the following possible inheritance patterns: Not inherited. More than one pattern can apply in different cases.
Related genes
Other names
Drug-induced Stevens Johnson syndrome
Lyell's syndrome
Mycoplasma-induced Stevens Johnson syndrome
Stevens-Johnson syndrome
Stevens-Johnson syndrome toxic epidermal necrolysis spectrum
Toxic epidermal necrolysis
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For further information, ... Two severe reactions which can develop are called Stevens-Johnson syndrome and toxic epidermal necrolysis. Whilst there are some differences in the features of these two conditions, toxic epidermal necrolysis can be considered a more widespread form of Stevens-Johnson syndrome. Toxic epidermal necrolysis can be.
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If you feel unwell, co... Important precautions Severe Skin Reactions Stop taking Bactrim immediately and seek urgent medical help if you develop a skin rash, peeling, or mouth sores. These can be signs of rare but life-threatening skin reactions (Stevens-Johnson syndrome or toxic epidermal necrolysis). Pregnancy (Third Trimester) Do not take.
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Further reading and references
- Stevens-Johnson syndrome/toxic epidermal necrolysis; MedlinePlus, National Library of Medicine.
- Amstutz U, Shear NH, Rieder MJ, Hwang S, Fung V, Nakamura H, Connolly MB, Ito S, Carleton BC; CPNDS clinical recommendation group. Recommendations for HLA-B*15:02 and HLA-A*31:01 genetic testing to reduce the risk of carbamazepine-induced hypersensitivity reactions. Epilepsia. 2014 Apr;55(4):496-506. doi: 10.1111/epi.12564. Epub 2014 Mar 5.
- Cheng CY, Su SC, Chen CH, Chen WL, Deng ST, Chung WH. HLA associations and clinical implications in T-cell mediated drug hypersensitivity reactions: an updated review. J Immunol Res. 2014;2014:565320. doi: 10.1155/2014/565320. Epub 2014 May 8.
- Chung WH, Hung SI, Yang JY, Su SC, Huang SP, Wei CY, Chin SW, Chiou CC, Chu SC, Ho HC, Yang CH, Lu CF, Wu JY, Liao YD, Chen YT. Granulysin is a key mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and toxic epidermal necrolysis. Nat Med. 2008 Dec;14(12):1343-50. doi: 10.1038/nm.1884. Epub 2008 Nov 23.
- Chung WH, Hung SI. Recent advances in the genetics and immunology of Stevens-Johnson syndrome and toxic epidermal necrosis. J Dermatol Sci. 2012 Jun;66(3):190-6. doi: 10.1016/j.jdermsci.2012.04.002. Epub 2012 Apr 11.
- Lee HY, Chung WH. Toxic epidermal necrolysis: the year in review. Curr Opin Allergy Clin Immunol. 2013 Aug;13(4):330-6. doi: 10.1097/ACI.0b013e3283630cc2.
- Mockenhaupt M. Stevens-Johnson syndrome and toxic epidermal necrolysis: clinical patterns, diagnostic considerations, etiology, and therapeutic management. Semin Cutan Med Surg. 2014 Mar;33(1):10-6. doi: 10.12788/j.sder.0058.
- Ostrov DA, Grant BJ, Pompeu YA, Sidney J, Harndahl M, Southwood S, Oseroff C, Lu S, Jakoncic J, de Oliveira CA, Yang L, Mei H, Shi L, Shabanowitz J, English AM, Wriston A, Lucas A, Phillips E, Mallal S, Grey HM, Sette A, Hunt DF, Buus S, Peters B. Drug hypersensitivity caused by alteration of the MHC-presented self-peptide repertoire. Proc Natl Acad Sci U S A. 2012 Jun 19;109(25):9959-64. doi: 10.1073/pnas.1207934109. Epub 2012 May 29.
- Sukasem C, Katsila T, Tempark T, Patrinos GP, Chantratita W. Drug-Induced Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Call for Optimum Patient Stratification and Theranostics via Pharmacogenomics. Annu Rev Genomics Hum Genet. 2018 Aug 31;19:329-353. doi: 10.1146/annurev-genom-083115-022324. Epub 2018 Apr 13.
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