Stevens–Johnson syndrome
From Wikipedia, the free encyclopedia
| Stevens–Johnson syndrome | |
|---|---|
| Classification and external resources | |
 Conjunctivitis in SJS |
|
| ICD-10 | L51.1 |
| ICD-9 | 695.1 |
| DiseasesDB | 4450 |
| MedlinePlus | 000851 |
| eMedicine | emerg/555 derm/405 |
| MeSH | D013262 |
Stevens–Johnson syndrome (SJS) 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 |
[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).
Both diseases can be mistaken for erythema multiforme. Erythema multiforme is sometimes caused by a reaction to a medication but is more often a 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.[1]
[edit] Epidemiology
SJS is a rare condition, with a reported incidence of around 2.6 per million people per year.[1] In the United States, there are about 300 new diagnoses per year.
[edit] Causes
SJS is thought to arise due to a disorder of the immune system.[1]
It can be caused by infections (usually following infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), adverse effects of drugs (allopurinol, diclofenac, etravirine, fluconazole,[2] valdecoxib, sitagliptin, oseltamivir, penicillins, barbiturates, sulfonamides, phenytoin, azithromycin, modafinil[3], lamotrigine, nevirapine,pyrimethamine,ibuprofen[4], ethosuximide, carbamazepine)[5][6], malignancy (carcinomas and lymphomas), or idiopathic factors (up to 50% of the time). SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng. SJS may also be caused by cocaine usage.[7]
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 Stevens–Johnson Syndrome, as well as 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 Stevens–Johnson syndrome occurring.
Non-steroidal anti-inflammatory drugs and medications against gout often cause SJS in adults, but rarely in children. 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.[1]
[edit] Genetics
In some East Asians studied (Han Chinese, 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.[8][9][10] A study in Europe suggested that the gene marker is only relevant for East Asians.[11][12] 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."[13]
[edit] Symptoms
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.[1]
[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.[1]
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.[1]
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.[1]
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 and impaired vision, as well as 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.[7] Other outcomes include organ damage/failure, cornea scratching and blindness.
[edit] Eponym
It is named for Dr. Albert Mason Stevens and Dr. Frank Chambliss Johnson, American pediatricians who jointly published a description of the disorder in 1922.[14][15][16]
[edit] Notable sufferers
- Padma Lakshmi, actress, model, television personality, and cookbook writer[17]
- Tessa Keller of MTV show Laguna Beach[18]
- Sabrina Brierton Johnson, whose family sued the manufacturer of Children's Motrin, Johnson & Johnson.[19]
[edit] See also
[edit] References
- ^ a b c d e f g h 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.
- ^ Medsafe Data Sheet March 8, 2005. Accessed April 26, 2007.
- ^ US FDA 2007 Safety Alerts for Drugs, Biologics, Medical Devices, and Dietary Supplements
- ^ Raksha MP, Marfatia YS (2008). "Clinical study of cutaneous drug eruptions in 200 patients". Indian J Dermatol Venereol Leprol 74 (1): 80. doi:. PMID 18193504.
- ^ 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:. PMID 11579247.
- ^ 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. PMID 16394456. http://www.ijdvl.com/article.asp?issn=0378-6323;year=2005;volume=71;issue=5;spage=325;epage=328;aulast=Devi.
- ^ a b Stevens–Johnson Syndrome - emerg/555 at eMedicine
- ^ Chung WH, Hung SI, Hong HS, et al. (April 2004). "Medical genetics: a marker for Stevens–Johnson syndrome". Nature 428 (6982): 486. doi:. PMID 15057820.
- ^ 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: 2087. doi:. PMID 18637831.
- ^ 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:. PMID 17509004.
- ^ 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:. PMID 16981842.
- ^ Lonjou C, Thomas L, Borot N, et al. (2006). "A marker for Stevens–Johnson syndrome ...: ethnicity matters". Pharmacogenomics J. 6 (4): 265–8. doi:. PMID 16415921.
- ^ 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:. PMID 18192896.
- ^ Stevens–Johnson syndrome at Who Named It?
- ^ 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.
- ^ Stevens–Johnson syndrome - Definitions from Dictionary.com
- ^ Jess Cartner-Morley, "Beautiful and Damned", The Guardian, 8 April 2006
- ^ Source: Daly, Melissa. "My Friend Ditched Me!" Seventeen Magazine Dec. 2006: 102
- ^ Jury finds for J&J in Motrin suit
[edit] External links
- Stevens Johnson Syndrome Foundation
- Association of victims of medicines
- SJS & Lyell Francophone Patient Association
- Book On Stevens Johnson Syndrome by a survivor
