2nd Pediatric Infectious Disease training course

2007

CASE ROUND 2

Prof. A. Gervaix

Clinical case • Justin, 1rst baby, pregnancy uneventful • Born by cesarean section at 40 weeks of gestation • Apgar score 9 ­ 9 ­ 9 • Weight + Height + HC within normal limits • No infectious risks • No peri/postnatal problems • Guthrie test: normal • Maternal breast feeding

• At 5 weeks of age Conjonctivitis of the left eye => good evolution under tx of Biocidan (antiseptic) drops for 2 days

• At 6 weeks of age Nasal obstruction + foul smell of the left ear => ED: no otitis Dx: Viral problem, symptomatic tx

• Following days: A small hyperemic lesion behind the left ear + red spots on the face, persistance of foul smell of the left ear.

• No fever • Increase of skin lesions with blisters and bullae • Hôpital de Fribourg => HUG

Clinical case: Justin

Clinical case: Justin

Anything else you would like to know ?

History 1. Any medication ? 2. Bullous diseases in the family ?

Clinical status 1. Mucosal involvement ?

Clinical case: Justin

Yes

No

No

Most likely diagnosis ?

Clinical case: Justin

Staphylococcal scalded skin syndrome

Staphylococcal scalded skin syndrome

Pathogenesis

PIGS study: Among 1363 nasal swabs taken in Swiss children in 2006 S. aureus was isolated in 572 cases (41.9%) 22 strains (3.9%) were positive for Exfoliatin A or B

Desmoglein 1 is the target for the exfoliative toxins (serine proteases) released by S. aureus

Staphylococcal scalded skin syndrome SSSS: superficial epidermal blisters Toxic epidermal necrolysis (drug reaction): subepidermal blisters with necrotic keratinocytes (mucosal involvement)

Staphylococcal scalded skin syndrome What makes the difference between

SSSS (generalized) and bullous impetigo (localized) ?

The immune status !

Staphylococcal scalded skin syndrome

• Anti­Exfoliatine antibodies: • Positive in 50% of individuals >10 years of age • Positive in 90% of individuals >40 years • BUT 0% in SSSS cases

• Most of cases of SSSS occur before the age of 5 y. • Mortality in neonates and infants < 5%, up to 60% in adults

• Epidemics in nursery and day cares (hand transmission)

Staphylococcal scalded skin syndrome

Management and treatment:

Lab exams:

WBC count, often within normal limits CRF often within normal limits Blood cultures are negative

Skin biopsy Swab at the site of infection + carriage sites

culture S. aureus and Exfo A/B production

Staphylococcal scalded skin syndrome Management and treatment:

Goals: 1) Eradication of S. aureus at the site of infection 2) Stop toxin production

Antibiotics (IV then oral): Clindamycin ± Flucloxacillin

Care of the skin as for burned patients (no local Abtx) Pain relief and hydration

Decolonisation: Nasal mupirocin and Hibiscrub® soap

Test and treat people around

Bibliography ­ Nelson Textbook of Pédiatris (17th edition) ­ Textbook of Pediatric Infectious Diseases (5th Edition) ­ Red Book (27th Edition) ­ Atlas of Pediatric Dermatology (Harper, Oranje, Prose; Blackwell Science) ­ Atlas of Pediatric Physical Diagnosis (4th Edition; Mosby) ­ Stanley et al. 2006, Pemphigus, Bullous Impetigo, and the Staphylococcal Scalded­Skin

Syndrome; N Engl J Med 2006;355:1800­10. ­ Todd, Staphylococcal Infections; Pediatrics in Review, Vol.26 No.12 Dec 2005 ­ Iwatsuki et al. 2006, Staphylococcal cutaneus infections: Invasion, evasion and aggression;

J Derm Science (2006) 42, 203­214 ­ Murray 2005, Recognition and management of Staphylococcus aureus toxin­mediated

disease; Internal Medicine Journal 2005; 35:S106­S119 ­ Ladhani et Evans 1998, Staphylococcal scalded skin syndrome; Arch Dis Child 1998;78:85­

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