(SJS) is a severe and life-threatening
Condition . It is a thought to be a
Hypersensitivity complex affecting the skin and the
Mucous Membrane s. 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.
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 followed by the sudden development of circular mucocutaneous lesions (''target lesions'') 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
Urticarial Plaque s. Cases involving greater than 30% of body surface area are termed
Toxic Epidermal Necrolysis Syndrome (TENS) or
Lyell's Syndrome .
The cause of SJS is either infections (usually following viral infections such as s and
Lymphoma s), or
Idiopathic (up to 50% of the time). SJS has also been consistently reported as an uncommon side effect of herbal supplements containing
Ginseng .
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.
SJS is fatal in approximately 5% of cases. The risk for death can be estimated using the SCORTEN scale, which takes a number of prognostic indicators into account
{Link without Title} . Other outcomes include organ damage and blindness.
