Steven-Johnson Syndrome
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Allergy Immunology subdivisionDepartment of PediatricsSumatera Utara University
Medical Faculty / H. Adam Malik Hospital
Steven-Johnson Syndrome
• Drug induced problem
• Found by Stevens & Johnson (1922)
• Acute, self-limited disease, but high
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• Acute, self-limited disease, but high morbidity and potentially life-threatening
Etiology
• Immune-complex-mediated hypersensitivity disorder
• May caused by : drugs, viral infection ( eg : HIV ) (eg:Mycoplasma pneumoniae)
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( eg : HIV ) (eg:Mycoplasma pneumoniae) & malignancies or idiopathic
• Predispose factors : autoimmune disorders ( SLE ), HLA-Linked, genetic
Etiology…………………• Drugs such as :
Carbamazepin (most)
DPT vaccine
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Phenoxymethylpenicilline
Amoxycilline
Metamizole natrium
Acetylsalicylate acid
Clinical Manifestations• Vesicobulous lesion :
Central Necrosis
Erythematous macules
Vesicles Bullae
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Area denudation : face, trunk & extremities• Severe purulent conjunctivitis• Severe stomatitis with extensive mucosal necrosis
LaboratoryNo specific laboratory abnormalities• Leucocytosis • Elevated erythrocyte sedimentation rate• Elevated liver transaminase levels• Decreased serum values
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• Decreased serum values• Skin biopsy
Management• Supportive & symptomatic
• Ophthalmologic consultation : occular-corneal scarring (vision loss andsymblepharon)
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• Oral lesion: mouthwashes & glycerin swabs
• Observed vaginal lesions
• Topical pain/anesthetic reliefer: dipenhydramine, dyclonine, viscous lidocaine
Management……………….• Compressed denude skin lessions with saline/burowi solution
• Antibiotic for secondary bacterial infections
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infections
• Corticosteroid (dexamethasone) : 1mg/kg/day (loading dose), then 0,2-0,5 mg/kg/day
Management ……………• May require admission to intensive care unit (ICU), IV fluids, nutritional support, wound & burn care, daily saline/burowi solution compress, parafin gauze or hydrogel dressing of denude areas, saline compress of eyelids/lips/nose, urinary
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compress of eyelids/lips/nose, urinary cathetherization
• Daily occular infection examination, systemic antibiotics (urinary/cutaneous infections).
• Bacterimiae may cause of death
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