1. Emergencies in Pediatric Dermatology Ayelet Shani Adir, M.D. Pediatric Dermatologist Haemek Medical Center

2. Widespread skin involvement (massive trans-epidermal loss) Skin findings as clues for diagnosis Dermatology in Emergency Medicine

3. Case 1 15 months old healthy infant Fever, malaise and skin tenderness for the past 3 days

4. SSSS Staphylococcal Scalded Skin Syndrome Occurs mainly in infants and young children (<5 years) Malaise, fever, generalized macular erythema with tenderness of skin Exfoliation with exudation and crusting around mouth, eyes and nose Diagnosis- Tzanck smear and bacterial cultures

5. SSSS: Clinical Findings Generalized erythema with flexural accentuation Skin tenderness Flaccid bulla in skin folds Positive Nikolsky’s sign Desquamation

6. SSSS: Therapy Maintain fluid status Intravenous anti- staphylococcal antibiotics Prevent secondary infection

7. Case 2 11 year old healthy girl Generalized skin eruption with bullae Two weeks before: UTI treated with Resprim for 8 days, later switched to Zinnat

8. Physical Examination Febrile, 39 0 C Generalized maculo-papular rash on skin, erosions on oral mucosa and eyes Large tense bulla on skin

9. Toxic Epidermal Necrolysis Nikolsky’s Sign

10. SJS and TEN Incidence: 0.4-1.2 cases per million per year SJS: detachment of <10% BSA with widespread targetoid lesions SJS-TEN overlap: 10-30% detachment TEN: >30% detachment

11. SJS and TEN Prodromal period of 1-14 days with fever, cough, coryza, sore throat, myalgia Extensive erosions of mucous mebranes: oropharynx, eyes, genitalia, rectum Variable skin involvement

12. SJS-TEN Therapy Prompt discontinuation of suspected drug PICU Wound care: nonstick dressings, debridement Ophthalmologic intervention Hydration, electrolyte balance, nutritional support, pain control

13. SJS-TEN Therapy Use of systemic corticosteroids is controversial High-dose IVIG (inhibit Fas- mediated apoptosis) 0.4-2 gr/kg/d for 2-5 days

14. Hypersensitivity Syndrome Drug Rash with Eosinophila and Systemic Symptoms (DRESS Syndrome) Severe multi-organ reaction Fever, rash, Lymphadenopathy and hepatitis Anticonvulsants, sulfa antibiotics 1:10000 exposures

15. Dress Syndrome Immediate withdrawal of suspected drug Close monitoring of liver and kidney functions Corticosteroids 1-2 mg/kg IVIG

16. Skin Findings as Clues for Diagnosis Systemic infections Kawasaki’s Disease Child Abuse purpuric, necrotic lesions of Meningococcemia Condyloma acuminata

17. תודה רבה