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Ri
Stevens-Johnson syndrome
severe expression of erythema multiforme involves the skin and the mucous membranes
oral, nasal, eye, vaginal, urethral, GI, and lower respiratory tract mucous membranes
Stevens-Johnson syndrome
Etiologic categories
25-50% of cases
Stevens-Johnson syndrome
Drugs and malignancies induced are most often Related more often due to infections
Childs
Stevens-Johnson syndrome
Early spring and winter Male-to-female ratio is 2:1 Caucasian predominance has been reported Mortality rate : 3-15% (or 30%) second to fourth decade
Stevens-Johnson syndrome
Stevens-Johnson syndrome
Stevens-Johnson syndrome
Fever Epistaxis Tachycardia, hypotension Conjunctivitis, corneal ulcerations Erosive vulvovaginitis or balanitis Altered level of consciousness, Seizures, coma
Ocular symptoms
History
1 to 14 days fever, sore throat, chills, headache, vomiting, diarrhea and malaise may be present
History
last 2-4 weeks. typically are nonpruritic may lead to mucosal scarring and loss of function of the involved organ system
History
Esophagus involvement
Esophageal strictures
Respiratory failure Corneal ulceration Anterior uveitis Keratitis or panophthalmitis (3-10%blindness)
Ocular sequelae
History
The rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques, or confluent erythema.
The center of these lesions may be vesicular, purpuric, or necrotic. The typical lesion has the appearance of a target.
The target lesion exhibits central necrosis surrounded by a rim of perivenular inflammation
Mucosal involvement may include erythema, edema, sloughing, blistering, ulceration, and necrosis.
History
Although lesions may occur anywhere, but the rash may be confined to any one area of the body
Lesions may become bullous and later rupture, leaving denuded skin.
History
Penicillins Sulfas Phenytoin (and related anticonvulsants) Carbamazepine Barbiturates valdecoxib (COX-2 inhibitor)
Penicillin, sulfas, or phenytoin, had previously been prescribed to more than 2/3 of all patients with SJS.
herpes simplex virus (HSV) Influenza Mumps cat-scratch fever (Bartonella henselae) mycoplasma lymphogranuloma venereum Histoplasmosis cholera In children, Epstein-Barr virus and enteroviruses have been identified
TNF-a IL-2 receptor IL-6 CRP However, none of these serologic tests is used routinely in diagnosing SJS
Normal WBC count or a nonspecific leukocytosis A severely elevated WBC count indicates a superimposed bacterial infection
blister formation
airway and hemodynamic stability fluid status, wound/burn care Electrolyte correction pain control
Offending drugs must be stopped Underlying diseases and secondary infections must be identified and treated Oral lesions
Useful in high doses early in the reaction But morbidity and mortality actually may increase in association with steroid use
Because of increasing secondary infection rate Some authors believe that they are contraindicated