1. STEVENS JOHNSON
SYNDROME

2. What is it?
• Stevens-Johnson syndrome (SJS) is an immune-complex–mediated
hypersensitivity reaction that is a severe expression of erythema multiforme
• It is known by some as erythema multiforme major
• It involves the skin AND the mucous membranes
• Cell death with separation of epidermis from dermis
• Significant involvement of oral, nasal, eye, vaginal, urethral, GI, and lower
respiratory tract mucous membranes may develop. GI and respiratory
involvement may progress to necrosis
• The features of SJS may be indistinguishable or overlap with the related
skin condition toxic epidermal necrolysis (TEN). Most dermatologists
diagnose TEN if more than 30% of body surface area is affected by
denuding skin.

3. MUCOUS MEMBRANE
INVOLVEMENT

4. • When BSA (body surface area) sloughing is less than 10%, the
mortality rate is 1-5%.
If more than 30% BSA sloughing is present, the mortality rate is
between 25% and 35%
• Lesions may continue to erupt in crops for as long as 2-3 weeks.
Mucosal scarring and loss of function of the involved organ system
• Esophageal strictures may occur when extensive involvement of the
esophagus exists
• Mucosal shedding in the tracheobronchial tree may lead to
respiratory failure.

5. Aetiology(1) INFECTIOUS
– Viral diseases: herpes simplex virus (HSV), AIDS, coxsackie viral infections, influenza,
hepatitis, mumps,
– Bacterial: Group A beta streptococci, diphtheria, Brucellosis, Mycoplasma pneumoniae and
typhoid.
– Protozoa: Malaria and trichomoniasis
– In children, Epstein-Barr virus and enteroviruses have been identified.
– More than half of the patients with SJS report a recent upper respiratory tract infection
(2) DRUG INDUCED
– Penicillins and antibiotics (2/3 of patients with SJS)
– Anticonvulsants have been implicated : carbamazepine and phenytoin (most
anticonvulsant-induced SJS occurs in the first 60 days of use)
– NSAIDS
– Allopurinol
(3) MALIGNANCY RELATED
– Various carcinomas and lymphomas have been associated
(4) IDIOPATHIC
– SJS is idiopathic in 25-50% of cases
Paediatric cases are related more often to infections than to malignancy or a reaction

6. Who is at risk?
• Race: Caucasian predominance
• Sex: Male:female 2:1.
• Age: 20-40 year olds
(cases have been reported in children as
young as 3 months)

7. HISTORY
• Nonspecific upper respiratory tract infection
• Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-
4 weeks. The lesions are typically non-pruritic
• Fever occurs in up to 85% of cases
• Involvement of oral and/or mucous membranes may be severe enough that
patients may not be able to eat or drink
• Conjunctivitis
• Patients with genitourinary involvement may complain of dysuria or an
inability to void
• A history of a previous outbreak of SJS or of erythema multiforme may be
elicited.

8. EXAMINATION
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 is considered pathognomonic
Rings of red, white and pink
In contrast to the typical erythema multiforme lesions, these lesions have only
two zones of colour
Lesions may become bullous and later rupture, leaving denuded skin. Skin is
susceptible to secondary infection
The palms, soles, dorsum of the hands, and extensor surfaces are commonly
affected
The rash (symmetrical) may be confined to any one area of the body, most often the
trunk

9. Differentials
Burns, Chemical
Burns, Ocular
Burns, Thermal
Dermatitis, Exfoliative
Erythema Multiforme
Staphylococcal Scalded Skin Syndrome
Toxic Epidermal Necrolysis
Toxic Shock Syndrome

10. Investigations
No laboratory studies (other than biopsy) exist that
can aid the physician in establishing the
diagnosis
Skin biopsy is the definitive diagnostic study
Bullae are subepidermal
Epidermal cell necrosis may be noted
Perivascular areas are infiltrated with lymphocytes

11. TREATMENT
Treatment is primarily supportive and symptomatic.
Manage oral lesions with mouthwashes.
Topical anesthetics are useful in reducing pain and allowing the patient to
take in fluids.
Areas of necrotic skin must be covered
Antibiotics
Treat as patients with thermal burns.
Withdrawal of the suspected offending agent (Timing of withdrawal has been
linked to outcome)
Special attention to airway and haemodynamic stability, fluid status,
wound/burn care, and pain control
The use of systemic steroids is controversial. Treatment with systemic steroids
has been associated with an increased prevalence of complications.

12. Prognosis
Individual lesions typically should heal within 1-2
weeks
Most patients recover without problems
Development of serious sequelae, such as
respiratory failure, renal failure, and blindness,
determines prognosis in those affected
Up to 15% of all patients with SJS die as a result
of the condition

13. Extensive sloughing of epidermis
from Stevens Johnson syndrome.