Steven Jhonson Syndrome

Drug induced problem

Found by Stevens & Johnson (1922)

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) & malignancies or idiopathic

Predispose factors : autoimmune disorders (SLE), HLA-Linked, genetic

Drugs such as :

Carbamazepin (most)

DPT vaccine

Phenoxymethylpenicilline

Amoxycilline

Metamizole natrium

Acetylsalicylate acid

Clinical Manifestations

Vesicobulous lesion :

Area denudation : face, trunk & extremities

Severe purulent conjunctivitis

Severe stomatitis with extensive mucosal necrosis

Laboratory

No specific laboratory abnormalities

Leucocytosis

Elevated erythrocyte sedimentation rate

Elevated liver transminase levels

Decreased serum values

Skin biopsy

Management

Supportive & symptomatic

Ophthalmologic consultation : occular-corneal scarring (vision loss and symblepharon)

Oral lesion: mouthwashes & glycerin swabs

Observed vaginal lesions

Topical pain/anesthetic reliefer: dipenhydramine, dyclonine, viscous lidocaine

Compressed denude skin lessions with saline/burowi solution

Antibiotic for secondary bacterial infections

Corticosteroid (dexamethasone) : 1mg/kg/day (loading dose), then 0,2-0,5 mg/kg/day

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 cathetherization

Daily occular infection examination, systemic antibiotics (urinary/cutaneous infections). Bacterimiae may cause of death

 

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