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Stevens-Johnson 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...

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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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Stevens-Johnson syndrome with diffuse esophageal involvement - Esophagoscopy Clinic - Brief Article
From Ear, Nose & Throat Journal, 4/1/02 by Peter C. Belafsky

A 34-year-old man with no significant medical history came to us with complaints of a rash, oropharyngeal ulcers, and severe odynophagia. The onset of his symptoms occurred 2 days after he had taken amoxicillin for a sinus infection.

Physical examination revealed that the man was febrile and slightly dehydrated. He had severe blistering in his oral cavity and on his lips, and the ventral surface of his tongue was desquamated (figure 1). Transnasal esophagoscopy (TNE) revealed severe sloughing of the pharyngeal mucosa and extensive desquamation of the cervical esophagus (figure 2). All biopsy and culture results were negative.

The patient was hospitalized and treated with aggressive rehydration, systemic corticosteroids to reduce inflammation, and intravenous antimicrobials and antivirals to prevent concomitant infection. He slowly improved over the next 21 days.

Follow-up TNE to ensure the absence of stricture formation was performed 8 weeks after his initial visit, and the findings were normal.

Acute manifestations of Stevens-Johnson syndrome within the esophagus may include bulla formation and erosive involvement of the mucosa with resultant hemorrhage. Complications can include mucosal scarring with esophageal stricture and web formation.

From the Center for Voice Disorders, Department of Otolaryngology, Wake Forest University Medical Center, Winston-Salem, N.C.; www.thevoicecenter.org

COPYRIGHT 2002 Medquest Communications, LLC
COPYRIGHT 2002 Gale Group

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