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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