Stevens-Johnson syndrome
| Stevens-Johnson syndrome | |
| ICD-10 | L51.1 |
|---|---|
| ICD-9 | 695.1 |
| DiseasesDB | 4450 |
| MedlinePlus | 000851 |
| eMedicine | emerg/555 derm/405 |
| MeSH | D013262 |
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Overview
Stevens-Johnson syndrome (SJS) is a life-threatening condition affecting the skin, in which due to cell death the epidermis separates 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.
Classification
There is agreement in the medical literature that Stevens-Johnson syndrome can be considered a milder form of toxic epidermal necrolysis (TEN). However, there is debate whether it falls on a spectrum of disease that includes erythema multiforme and erythema multiforme major (also known as erythema multiforme majus).[1][2][3][4][5]
Epidemiology
SJS is a rare condition, with a reported incidence of around one case per million people per year.
Causes
SJS can be caused by infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), allergic reactions to drugs, (Dicloflex, fluconazole,[6] valdecoxib, penicillins, barbiturates, sulfas, phenytoin, Modafinil, lamotrigine, nevirapine, Ibuprofen[7], ethosuximide, carbamazepine)[8][9], 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.[10]
Although Stevens Johnson Syndrome may be caused by viral infections or malignancies, severe allergic reactions to medication is the leading cause. Medications that have traditionally been known to lead to Stevens Johnson Syndrome, Erythema multiforme, and Lyell's Syndrome (Toxic Epidermal Necrolysis) include;
- sulfonamides (antibiotics)
- penicillin's (antibiotics)
- barbiturates (sedatives)
- phenytoin - Dilantin (anticonvulsant)
Treatment
Discontinue all medications, particularly those known to cause SJS reactions. Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment (2002). Treatment with corticosteroids is controversial since it might aggravate the condition or increase risk of secondary infections. 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.
Prognosis
SJS proper (with less than 10% of body surface area involved) has the 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.[10] Other outcomes include organ damage and blindness.
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.[11][12][13]
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.
- ↑ Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC (1993). "Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme". Archives of dermatology. 129 (1): 92–6. doi:10.1001/archderm.129.1.92. PMID 8420497.
- ↑ Ogundele O, Silverberg MA. Erythema Multiforme. eMedicine.com. URL: http://www.emedicine.com/emerg/topic173.htm. Accessed on: May 6, 2007.
- ↑ Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC (2002). "Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study". Arch Dermatol. 138 (8): 1019–24. PMID 12164739. Unknown parameter
|month=ignored (help) - ↑ Medsafe Data Sheet March 8, 2005. Accessed April 26, 2007.
- ↑ Raksha MP, Marfatia YS (2008). "Clinical study of cutaneous drug eruptions in 200 patients". Indian J Dermatol Venereol Leprol. 74 (1): 80. 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:10.1097/00002030-200109280-00014. PMID 11579247.
- ↑ Devi K, George S, Criton S, Suja V, Sridevi P (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.
- ↑ 10.0 10.1 Stevens-Johnson Syndrome - emerg/555 at eMedicine
- ↑ Template:WhoNamedIt
- ↑ 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
External links