steven-johnson syndrome

Stevens–Johnson syndrome From Wikipedia, the free encyclopedia Jump to: navigation, search Stevens–Johnson syndrome Classification and external resources Person with Stevens–Johnson syndrome ICD-10 L51.1 ICD-9 695.13 OMIM 608579 DiseasesDB 4450 MedlinePlus 000851 eMedicine emerg/555 derm/405 MeSH D013262 Stevens–Johnson syndrome (SJS) is a form of erythema multiforme[1] which is a life-threatening condition affecting the skin in which cell death causes the epidermis to separate from the dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. Although the majority of cases are idiopathic, the main class of known causes is medications, followed by infections and (rarely) cancers. Contents [hide] * 1 Classification * 2 Signs and symptoms * 3 Causes o 3.1 Infectious o 3.2 Medication/drugs o 3.3 Genetics * 4 Treatment * 5 Prognosis * 6 Epidemiology * 7 History * 8 Notable cases * 9 See also * 10 References [edit] Classification There is agreement in the medical literature that Stevens–Johnson syndrome (SJS) can be considered a milder form of toxic epidermal necrolysis (TEN). These conditions were first recognised in 1922.[2] Both diseases can be mistaken for erythema multiforme. Erythema multiforme is sometimes caused by a reaction to a medication but is more often a type IV hypersensitivity reaction to an infection (caused most often by Herpes simplex) and is relatively benign. Although both SJS and TEN can also be caused by infections, they are most often adverse effects of medications. Their consequences are potentially more dangerous than those of erythema multiforme. [edit] Signs and symptoms Conjunctivitis (inflammation of eye and eyelid) in SJS SJS usually begins with fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient’s ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.[3] [edit] Causes SJS is thought to arise from a disorder of the immune system.[3] [edit] Infectious It can be caused by infections (usually following infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, mycoplasma pneumoniae or similar). [edit] Medication/drugs It can be caused by adverse effects of drugs (allopurinol, diclofenac, etravirine, Isotretinoin, aka Accutane, fluconazole,[4] valdecoxib, sitagliptin, oseltamivir, penicillins, barbiturates, sulfonamides, phenytoin, azithromycin, modafinil,[5] lamotrigine, nevirapine, pyrimethamine, ibuprofen,[6] ethosuximide, carbamazepine and gout medications).[7][8] Although Stevens–Johnson Syndrome can be caused by viral infections, malignancies or severe allergic reactions to medication, the leading cause appears to be the use of antibiotics and sulfa drugs. Medications that have traditionally been known to lead to SJS, erythema multiforme and toxic epidermal necrolysis include sulfonamides (antibiotics), penicillins (antibiotics), barbiturates (sedatives), lamotrigine and phenytoin (e.g. Dilantin) (anticonvulsants). Combining lamotrigine with sodium valproate increases the risk of SJS. Non-steroidal anti-inflammatory drugs are a rare cause of SJS in adults; the risk is higher for older patients, women and those initiating treatment.[2] Typically, the symptoms of drug-induced SJS arise within a week of starting the medication. People with systemic lupus erythematosus or HIV infections are more susceptible to drug-induced SJS.[3] SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng. SJS may also be caused by cocaine usage.[9] [edit] Genetics In some East Asian populations studied (Han Chinese and Thai), carbamazepine- and phenytoin-induced SJS is strongly associated with HLA-B*1502 (HLA-B75), an HLA-B serotype of the broader serotype HLA-B15.[10][11][12] A study in Europe suggested that the gene marker is only relevant for East Asians.[13][14] Based on the Asian findings, similar studies were performed in Europe which showed 61% of allopurinol-induced SJS/TEN patients carried the HLA-B58 (B*5801 allele – phenotype frequency in Europeans is typically 3%). One study concluded “even when HLA-B alleles behave as strong risk factors, as for allopurinol, they are neither sufficient nor necessary to explain the disease.”[15] [edit] Treatment SJS constitutes a dermatological emergency. All medications should be discontinued, particularly those known to cause SJS reactions. Patients with documented mycoplasma infections can be treated with oral macrolide or oral doxycycline.[3] Initially, treatment is similar to that for patients with thermal burns, and continued care can only be supportive (e.g. intravenous fluids and nasogastric or parenteral feeding) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer). Dermatologists and surgeons tend to disagree about whether the skin should be debrided.[3] Beyond this kind of supportive care, there is no accepted treatment for SJS. Treatment with corticosteroids is controversial. Early retrospective studies suggested that corticosteroids increased hospital stays and complication rates. There are no randomized trials of corticosteroids for SJS, and it can be managed successfully without them.[3] Other agents have been used, including cyclophosphamide and cyclosporine, but none have exhibited much therapeutic success. Intravenous immunoglobulin (IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptics, maintaining a warm environment, and intravenous analgesics. An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids, leading to corneal vascularization, impaired vision and a host of other ocular problems. Also, an extensive physical therapy program ensues after the patient is discharged from the hospital. [edit] Prognosis SJS proper (with less than 10% of body surface area involved) has a mortality rate of around 5%. The risk for death can be estimated using the SCORTEN scale, which takes a number of prognostic indicators into account.[9] Other outcomes include organ damage/failure, cornea scratching and blindness. [edit] Epidemiology Stevens-Johnson syndrome is a rare condition, with a reported incidence of around 2.6[3] to 6.1[2] cases per million people per year. In the United States, there are about 300 new diagnoses per year. The condition is more common in adults than in children. Women are affected more often than men, with cases occurring at a three to two sex ratio.[2] [edit] History Stevens-Johnson Syndrome is named for Albert Mason Stevens and Frank Chambliss Johnson, American pediatricians who in 1922 jointly published a description of the disorder in the American Journal of Diseases of Children.[16][17][18][19] [edit] Notable cases * Padma Lakshmi, actress, model, television personality, and cookbook writer;[20] * Tessa Keller of MTV show Laguna Beach;[21] * Sabrina Brierton Johnson, whose family unsuccessfully sued the manufacturer of Children’s Motrin, Johnson & Johnson, after a case of SJS blinded her.[22] * Manute Bol, former professional Basketball Player and member of NBA’s Washington Bullets, Golden State Warriors, Philadelphia 76ers, and Miami Heat. He died on June 19, 2010, of kidney failure.[23] [edit] See also * Erythema multiforme * List of SJS inducing substances * List of cutaneous conditions [edit] References 1. ^ Stevens-Johnson syndrome at Dorland’s Medical Dictionary 2. ^ a b c d Ward, K. E.; Archambault, R.; Mersfelder, T. L. (2010). “Severe adverse skin reactions to nonsteroidal antiinflammatory drugs: A review of the literature”. American Journal of Health-System Pharmacy 67: 206. doi:10.2146/ajhp080603. edit 3. ^ a b c d e f g Tigchelaar H, Kannikeswaran N and Kamat D (December 1, 2008). “Stevens-Johnson Syndrome: An Intriguing Diagnosis”. Consultant for Pediatricians. http://www.consultantlive.com/consultant-for-pediatricians/article/1145470/1403936. 4. ^ Medsafe Data Sheet March 8, 2005. Accessed April 26, 2007. 5. ^ US FDA 2007 Safety Alerts for Drugs, Biologics, Medical Devices, and Dietary Supplements 6. ^ Raksha MP, Marfatia YS (2008). “Clinical study of cutaneous drug eruptions in 200 patients”. Indian J Dermatol Venereol Leprol 74 (1): 80. doi:10.4103/0378-6323.38431. PMID 18193504. 7. ^ Fagot J, Mockenhaupt M, Bouwes-Bavinck J, Naldi L, Viboud C, Roujeau J (2001). “Nevirapine and the risk of Stevens–Johnson syndrome or toxic epidermal necrolysis”. AIDS 15 (14): 1843–8. doi:10.1097/00002030-200109280-00014. PMID 11579247. 8. ^ Devi K, George S, Criton S, Suja V, Sridevi P (1 September 2005). “Carbamazepine–the commonest cause of toxic epidermal necrolysis and Stevens–Johnson syndrome: a study of 7 years”. Indian J Dermatol Venereol Leprol 71 (5): 325–8. doi:10.4103/0378-6323.16782. PMID 16394456. http://www.ijdvl.com/article.asp?issn=0378-6323;year=2005;volume=71;issue=5;spage=325;epage=328;aulast=Devi. 9. ^ a b Stevens–Johnson Syndrome – emerg/555 at eMedicine 10. ^ Chung WH, Hung SI, Hong HS, et al. (April 2004). “Medical genetics: a marker for Stevens–Johnson syndrome”. Nature 428 (6982): 486. doi:10.1038/428486a. PMID 15057820. 11. ^ Locharernkul C, Loplumlert J, Limotai C, et al. (July 2008). “Carbamazepine and phenytoin induced Stevens–Johnson syndrome is associated with HLA-B*1502 allele in Thai population”. Epilepsia 49 (12): 2087. doi:10.1111/j.1528-1167.2008.01719.x. PMID 18637831. 12. ^ Man CB, Kwan P, Baum L, et al. (May 2007). “Association between HLA-B*1502 allele and antiepileptic drug-induced cutaneous reactions in Han Chinese”. Epilepsia 48 (5): 1015–8. doi:10.1111/j.1528-1167.2007.01022.x. PMID 17509004. 13. ^ Alfirevic A, Jorgensen AL, Williamson PR, Chadwick DW, Park BK, Pirmohamed M (September 2006). “HLA-B locus in Caucasian patients with carbamazepine hypersensitivity”. Pharmacogenomics 7 (6): 813–8. doi:10.2217/14622416.7.6.813. PMID 16981842. 14. ^ Lonjou C, Thomas L, Borot N, et al. (2006). “A marker for Stevens–Johnson syndrome …: ethnicity matters”. Pharmacogenomics J. 6 (4): 265–8. doi:10.1038/sj.tpj.6500356. PMID 16415921. 15. ^ Lonjou C, Borot N, Sekula P, et al. (February 2008). “A European study of HLA-B in Stevens–Johnson syndrome and toxic epidermal necrolysis related to five high-risk drugs”. Pharmacogenet. Genomics 18 (2): 99–107. doi:10.1097/FPC.0b013e3282f3ef9c. PMID 18192896. 16. ^ Stevens–Johnson syndrome at Who Named It? 17. ^ A. M. Stevens, F. C. Johnson. A new eruptive fever associated with stomatitis and ophthalmia; report of two cases in children. American Journal of Diseases of Children, Chicago, 1922, 24: 526-533. 18. ^ Stevens–Johnson syndrome – Definitions from Dictionary.com 19. ^ American Medical Association (1922). American journal of diseases of children. American Medical Association. pp. 526–. http://books.google.com/books?id=E90fAAAAIAAJ&pg=PA526. Retrieved 5 June 2010. 20. ^ Jess Cartner-Morley, “Beautiful and Damned”, The Guardian, 8 April 2006 21. ^ Source: Daly, Melissa. “My Friend Ditched Me!” Seventeen Magazine Dec. 2006: 102 22. ^ Jury finds for J&J in Motrin suit 23. ^ FanHouse Staff (19). “Manute Bol Dies at Age 47”. Fanhouse. http://nba.fanhouse.com/2010/06/19/manute-bol-dies-acute-kidney-failure/. Retrieved 20 June 2010.

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