Stevens-Johnson Syndrome

Approach to SJS; a form of severe toxic epidermal necrolysis.

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. 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).

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

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

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

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

8. 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)

9. 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)

10. History C utaneous lesions develops abruptly: typically are non-pruritic, but are p ainful hemorrhagic erosions The rash begin as macules; develops into vesicles , bullae. Later rupture, leaving denuded skin. S usceptible to secondary infection

11. Investigations F BC may reveal Normal WBC count or leukocytosis Highly elevated WBC count indicates a superimposed bacterial infection

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

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

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

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

16. 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.