Stevens-Johnson syndrome (SJS)

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

potential for severe morbidity and even death.

Stevens-Johnson syndrome

Etiologic categories

Infection Drug-induced Malignancy-related Collagen vascular disease Idiopathic

25-50% of cases

Stevens-Johnson syndrome

Adults and the elderly

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

have been reported as young as 3 months

Stevens-Johnson syndrome

Individuals appear to be more susceptible to developing SJS

HLA-Bw44 A part of HLA-B12 HLA-DQB1*0601

Stevens-Johnson syndrome

Typical symptoms are as follows:

Cough productive of a thick purulent sputum Headache Malaise Arthralgia

Stevens-Johnson syndrome

The following signs may be noted on exam:

Fever Epistaxis Tachycardia, hypotension Conjunctivitis, corneal ulcerations Erosive vulvovaginitis or balanitis Altered level of consciousness, Seizures, coma

Ocular symptoms

Red eye Tearing Dry eye Pain Itching

Foreign body sensation Decreased vision Burn sensation Photophobia Diplopia

History

Typically begins with a nonspecific upper respiratory tract infection

1 to 14 days fever, sore throat, chills, headache, vomiting, diarrhea and malaise may be present

History

Mucocutaneous lesions develop abruptly

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)

Tracheobronchial mucosa shedding

Ocular sequelae

Vaginal stenosis and penile scarring Renal complications (rare)

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

most often the trunk.

Lesions may become bullous and later rupture, leaving denuded skin.

The skin becomes susceptible to secondary infection Fever or localized worsening

Fever occur in up to 85% of cases

History

Recurrences may occur if the responsible agent is not eliminated or reexposed

Drug etiologies include

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.

Hypersensitivity reaction to drugs has been suggested that probably both

Immune hypersensitivity reactions Mediated by toxic intermediates

Infectious diseases have been reported

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

Elevated in serum level :

TNF-a IL-2 receptor IL-6 CRP However, none of these serologic tests is used routinely in diagnosing SJS

CBC may reveal

Normal WBC count or a nonspecific leukocytosis A severely elevated WBC count indicates a superimposed bacterial infection

Typical histopathologic findings

Initially, dermal inflammatory cell infiltrate

superficial and mostly perivascular

Migration of lymphocytes along the dermoepidermal junction

Keratinocytes undergo apoptosis

Full-thickness necrosis of epidermis

blister formation

Conjunctival biopsy active ocular disease

subepithelial plasma cells & lymphocyte infiltration Lymphocytes are perivascular

The predominant is the helper T cell

Skin lesions are treated as thermal burns Attention to

airway and hemodynamic stability fluid status, wound/burn care Electrolyte correction pain control

Treatment of SJS is primarily supportive and symptomatic

Offending drugs must be stopped Underlying diseases and secondary infections must be identified and treated Oral lesions

mouthwashes Topical anesthetics

Useful in reducing pain Allowing the patient to take in fluids

Areas of denuded skin

Covered with compresses of saline

The systemic steroids use is controversial

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