Stevens–Johnson syndrome
| Stevens–Johnson syndrome | |
|---|---|
| Specialty | Dermatology  |
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.
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. In the United States, there are about 300 new diagnoses 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, sitagliptin, penicillins, barbiturates, sulfas, phenytoin, Azithromycin, Modafinil [7][citation needed], lamotrigine, nevirapine, Ibuprofen[8], ethosuximide, carbamazepine)[9][10], 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.[11]
Although Stevens Johnson Syndrome can be caused by viral infections, malignancies or severe allergic reactions to medication,the leading cause appears to be the inappropriate use of antibiotics and sulfa drugs. Medications that have traditionally been known to lead to Stevens Johnson Syndrome, Erythema Multiforme, Lyell's Syndrome, and Toxic Epidermal Necrolysis include sulfonamides (antibiotics), penicillins (antibiotics), barbiturates (sedatives), lamotrigine (anticonvulsant), and phenytoin - Dilantin (anticonvulsant). Combining lamotrigine with sodium valproate increases the risk of Stevens-Johnson Syndrome occurring.
Genetics
In East Asians the Carbamazepine and phenytoin induced syndrome is strongly associated with HLA-B75 (B*1502 allele of HLA-B).[12][13][14] A study in Europe suggested that the gene-marker is only relevant for East Asians.[15][16] Based on the Asian findings similar studies in Europe were performed, sixty-one percent of allopurinol-induced SJS/TEN carried the HLA-B58 (B*5801 allele - Phenotype frequency in Europeans is typically 3%), indicating that while the risk alleles differ between ethnicities, the HLA-B locus is strongly associated or closely linked to a strongly associated gene.[17]
Treatment
All medications should be discontinued, 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.
Recent studies conducted by the Natural Therapies Research Board indicate pharmaceutical grade essential plant oils of Lavender, Birch, Eucalyptus, Geranium and Helichrysum exhibit therapeutic success and improve symptoms.
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.[11] 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.[18][19][20]
People with Stevens-Johnson Syndrome
- Padma Lakshmi, actress, model, television personality, and cookbook writer[21]
- Tessa Keller of MTV show Laguna Beach[22]
- Sebastian Tamayo -- Donald Trump's Assistant
- Sabrina Brierton Johnson, whose family sued the manufacturer of Children's Motrin, Johnson & Johnson.[23]
- Seth O'Neill, Of Detroit Lakes, Minnesota.
References
- ^ Carrozzo M, Togliatto M, Gandolfo S (1999). "[Erythema multiforme. A heterogeneous pathologic phenotype]". Minerva Stomatol. 48 (5): 217–26. PMID 10434539.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Farthing P, Bagan J, Scully C (2005). "Mucosal disease series. Number IV. Erythema multiforme". Oral Dis. 11 (5): 261–7. PMID 16120111.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ 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.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ 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.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ 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. 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.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ 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.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ a b Stevens-Johnson Syndrome - emerg/555 at eMedicine
- ^ Chung WH, Hung SI, Hong HS; et al. (2004). "Medical genetics: a marker for Stevens-Johnson syndrome". Nature. 428 (6982): 486. doi:10.1038/428486a. PMID 15057820.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Locharernkul C, Loplumlert J, Limotai C; et al. (2008). "Carbamazepine and phenytoin induced Stevens-Johnson syndrome is associated with HLA-B*1502 allele in Thai population". Epilepsia. doi:10.1111/j.1528-1167.2008.01719.x. PMID 18637831.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Man CB, Kwan P, Baum L; et al. (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.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Alfirevic A, Jorgensen AL, Williamson PR, Chadwick DW, Park BK, Pirmohamed M (2006). "HLA-B locus in Caucasian patients with carbamazepine hypersensitivity". Pharmacogenomics. 7 (6): 813–8. doi:10.2217/14622416.7.6.813. PMID 16981842.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ 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.
{{cite journal}}: Explicit use of et al. in:|author=(help)CS1 maint: multiple names: authors list (link) - ^ Lonjou C, Borot N, Sekula P; et al. (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.
{{cite journal}}: Explicit use of et al. in:|author=(help); Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ 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