Stevens-Johnson syndrome
From Wikipedia, the free encyclopedia
| ICD-10 | L51.1 |
|---|---|
| ICD-9 | 695.1 |
| DiseasesDB | 4450 |
| MedlinePlus | 000851 |
| eMedicine | emerg/555 |
| MeSH | D013262 |
Stevens-Johnson syndrome (SJS) is a severe and life-threatening condition. It is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. SJS has been classified as a severe expression of erythema multiforme (EM), and is sometimes referred to as erythema multiforme major. This terminology is not consistent; medical texts often distinguish between causes of SJS, referring to drug induced syndrome as SJS and applying the term erythema multiforme to viral etiology.
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[edit] Epidemiology
SJS is a rare condition, with a reported incidence of around one case per million people per year.
[edit] Symptoms
SJS is characterized by a flu-like prodromal period of fever, sore throat, and headache followed by the sudden development of circular mucocutaneous lesions (target lesions) that can cover the majority of the skin. These lesions begin as macules and can develop into papules, vesicles, blisters, or urticarial plaques. Cases involving greater than 30% of body surface area are termed Toxic Epidermal Necrolysis Syndrome (TENS) or Lyell's Syndrome.
[edit] 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, (valdecoxib, penicillins, barbiturate, sulfas, phenytoin, lamotrigine, nevirapine,Ibuprofen, ethosuximide)[1][2], 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. [3]
[edit] 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. 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 if eyes are involved. Also, an extensive physical therapy program ensues after the patient is discharged from the hospital
[edit] Prognosis
Approximately 1 in 3 people survive from SJS. The risk for death can be estimated using the SCORTEN scale, which takes a number of prognostic indicators into account [4]. Other outcomes include organ damage and blindness.
