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

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

Erythema
multiforme

Severe
Cutaneous
Adverse
Reaction

SJS

SJS/TEN

TEN

SJS : with bullae, + mucous membrane

involvement
when <10% is called Steven Johnson
Syndrome
when 10-30% bullae called Steven
Johnson Syndrome-Toxic EpidermalNecrolysis (SSJ-TEN)
when the bullae> 30% is called Toxic
Epidermal Necrolysis (TEN).

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

 SKIN BIOPSY: non-inflammatory superficial

splitting of the epidermis
Blood Culture

Stevens-Johnson syndrome
Etiology
Drug-induced (60%)
Infection (20%)
Idiopathic (20%)

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

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. ( +
Nikolskys sign)

 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)

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

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

 Histological analysis of Skin Biopsy

under direct immunofluorescence
Typical full- thickness epidermal
necrolysis.
Due to extensive keratinocyte apoptosis.

full-thickness epidermal necrosis and

separation of dermis and epidermis

full-thickness epidermal necrosis and

separation of dermis and epidermis

necrotic keratinocytes within the entire epidermis and

vacuolar degeneration at the dermal-epidermal junction
resulting in subepidermal separation of the epidermis.

 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.