Stevens-Johnson syndrome is a life-threatening skin condition characterized by extensive necrosis and separation of the epidermis from the dermis. It exists on a spectrum of severity from Steven Johnson Syndrome (<10% body surface area involvement) to Stevens Johnson Syndrome-Toxic Epidermal Necrolysis (10-30% involvement) and Toxic Epidermal Necrolysis (>30% involvement). It is typically caused by hypersensitivity drug reactions or infections and presents with painful erosions and blistering of the skin and mucous membranes that can lead to vision loss, respiratory failure, or death in severe cases. Treatment focuses on stopping the offending agent, supportive care in intensive care units, wound care of d

1. Stevens-Johnson syndrome
• A form of TEN, life- threatening skin condition,
medical emergency.
• Extensive widespread necrosis, causing
epidermis to separate from the dermis.
By: Arravindh Vivekananthan

2. Pathophysiology
Hypersensitivity reaction
• Type III (IC rxn)
• Type IV ( cytotoxic CD8+ T lymphocyte)

3. Severe
Cutaneous
Adverse Reaction
Erythema
multiforme
SJS
SJS/TEN
TEN

4. SJS : with bullae, + mucous membrane
involvement
when <10% is called Steven Johnson
Syndrome
when 10-30% bullae called Steven Johnson
Syndrome-Toxic Epidermal-Necrolysis (SSJ-
TEN)
when the bullae> 30% is called Toxic
Epidermal Necrolysis (TEN).

12. SSSS
sparing of mucous membranes and risk factors,
such as drug history and clinical suspicion of staphylococcal infection.

13. • SKIN BIOPSY: non-inflammatory superficial
splitting of the epidermis
• Blood Culture

15. Stevens-Johnson syndrome
• Etiology
– Drug-induced (60%)
– Infection (20%)
– Idiopathic (20%)

17. – Infections
Paeds : EBV, enterovirus, URTI
Viral : HSV, HIV, mumps
Bacterial : Group A B-Haemolytic, diphteria,
M.pneumonie
Fungal : coccidioidomycosis, dermatophytosis,
and histoplasmosis

18. Clinical Manifestation
• Prodromal symptoms (1- 14 days):
– Non- specific symptoms : fever, headache, sore
throat, cough, malaise and/or burning of the eyes
followed by the appearance of mucocutaneous
lesions.
– Mucous membrane
– Diffuse rash, flaccid blistering. ( + Nikolsky’s sign)

19. • Ocular sequelae
– Corneal ulceration, anterior uveitis, blepharitis
– Vision loss, severe dry eye ( 1-3%)
• Esophagus, small bowel, colon involvement
– Esophageal strictures, impair enteral nutrition,
absorption of oral medications.
• Tracheobronchial mucosa shedding
– Respiratory failure 20% mechanical ventilation
• Vaginal stenosis and penile scarring
• PTSD in survivors
• Renal complications (rare)

21. History
• Cutaneous lesions develops abruptly:
– typically are non-pruritic, but are painful
hemorrhagic erosions
• The rash begin as macules; develops into
vesicles, bullae.
• Later rupture, leaving denuded skin.
– Susceptible to secondary infection

23. Investigations
• FBC may reveal
– Normal WBC count or leukocytosis
– Highly elevated WBC count indicates a
superimposed bacterial infection

24. • Histological analysis of Skin Biopsy under
direct immunofluorescence
– Typical full- thickness epidermal necrolysis.
– Due to extensive keratinocyte apoptosis.

25. full-thickness epidermal necrosis and separation
of dermis and epidermis

26. full-thickness epidermal necrosis and separation
of dermis and epidermis

27. necrotic keratinocytes within the entire epidermis and vacuolar degeneration
at the dermal-epidermal junction resulting in subepidermal separation of the
epidermis.

28. • Offending drugs must be stopped.
• Refer to Burn Units/ ICU. Warm environment, I/V analgesics.
• Supportive management, nutrition.
• I/V fluids with 0.7mL/kg per % of BSA
• NG/ parenteral feeding.
• Oral lesions : Analgesic mouth rinse for mouth ulcer.
• Ocular involvement : referral to ophthalmologist
( ophthalmic steroid/ local antibiotics)
• Denuded areas : non-adhesive dressings with silver nitrate.