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)

Severe Cutaneous

Adverse Reaction

Erythema multiforme

SJS

SJS/TEN

TEN

SJS : with bullae, + mucous membrane involvement

when <10% is called Steven Johnson Syndromewhen 10-30% bullae called Steven Johnson

Syndrome-Toxic Epidermal-Necrolysis (SSJ-TEN)when the bullae> 30% is called Toxic Epidermal

Necrolysis (TEN).

SSSSsparing 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%)

– InfectionsPaeds : EBV, enterovirus, URTIViral : HSV, HIV, mumpsBacterial : Group A B-Haemolytic, diphteria, M.pneumonieFungal : 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. ( + Nikolsky’s 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.