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(SJS) is a severe and life-threatening
Condition . It is thought to be a
Hypersensitivity complex affecting the skin and the
Mucous Membrane s.
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 ;
12 many consider erythema multiforme (EM) to be unrelated to SJS and TEN.
3Ogundele O, Silverberg MA. Erythema Multiforme. eMedicine.com. URL:
http://www.emedicine.com/emerg/topic173.htm . Accessed on: May 6, 2007.
Some classify SJS as a severe expression of
Erythema Multiforme , and it occasionally referred to as .
SJS is a rare condition, with a reported incidence of around one case per million people per year.
SJS is characterized by a flu-like
Prodromal period of fever, sore throat, and headache, which may also be accompanied by photophobia and redness in the eyes, followed by the sudden development of circular
Mucocutaneous Lesion s that can cover the majority of the skin. These lesions begin as
Macule s and can develop into
Papule s,
Vesicle s,
Blister s, or
Urticaria .
Stevens-Johnson syndrome is usually defined to refer to those cases where less than 10% of body surface is involved. Cases involving greater than 30% of body surface area are termed
Toxic Epidermal Necrolysis Syndrome (TENS) or
Lyell's Syndrome . Intermediate cases (10-30 percent) are called SJS/TEN overlap.
SJS can be caused by infections (usually following viral infections such as ,
Penicillin s,
Barbiturate s,
Sulfa s,
Phenytoin ,
Lamotrigine ,
Nevirapine ,
Ibuprofen ,
Ethosuximide ,
Carbamazepine )
45, malignancy (
Carcinoma s and
Lymphoma s), 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.Stevens-Johnson Syndrome -
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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
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.
Other outcomes include organ damage and blindness.
It is named for Albert Stevens and Frank Johnson.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.
- Padma Lakshmi , actress, model, television personality, and cookbook writerJess Cartner-Morley, "Beautiful and Damned", The Guardian, 8 April 2006
- Tessa Keller of MTV's hit show Laguna Beach
