Lyell's syndrome

Stevens-Johnson syndrome (SJS) is a severe and potentially life-threatening (15% of cases) disease, it is a hypersensitivity complex affecting the skin and the mucous membranes, a severe expression of erythema multiforme (EM) (and so SJS is also called erythema multiforme major). more...

Symptoms

SJS is characterized by fever, sore throat, and headache leading to 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. The most extreme cases are termed Toxic Epidermal Necrolysis Syndrome (TENS) or Lyell's Syndrome, in these cases the entire skin is affected.

Treatment

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). An ophthalmologist should be consulted if eyes are involved. Treatment with corticosteroids is controversial since it might aggravate the condition.

Cause

The cause of SJS is either infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), drug-induced (valdecoxib, penicillins, barbiturate, sulfas, phenytoin, lamotrigine, nevirapine), malignancy-related (carcinomas and lymphomas), or idiopathic (50% of the time)

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