Staphylococcal Scalded Skin Syndrome (SSSS)

Snapshot

• A 3-year-old girl presents to her pediatrician with a widespread rash. She recently had fevers and a sore throat. She has perioral fissues and a desquamating rash in the groin and neck. When light pressure is applied, the skin easily sloughs off. Superficial wound cultures and bacterial cultures are negative.

Introduction

• Clinical definition
  • exfoliative skin infection caused by Staphylococcus aureus toxins 
• Epidemiology
  • incidence
    • 9-25 per 100,000 children
  • demographics
    • affects neonates and children
• Pathophysiology
  • mechanism of injury
    • systemic spread of Staphylococcus aureus toxins exfoliative exotoxins A (ETA) or B (ETB) 
      • both are trypsin-like serine proteases that digest desmoglein-1, a cadherin that mediates keratinocyte adhesion in the epidermis
      • loss of cell-cell adhesion in stratum granulosum leads to bullae and sloughing
• Associated Staphylococcus aureus conditions
  • children
    • preceding respiratory tract infection
    • preceding conjunctivitis
    • preceding otitis media
• Prognosis
  • survival with treatment
• very good

Presentation

• Symptoms
  • primary symptoms
    • prodrome with irritability, malaise, fever, and sore throat
• Physical exam 
  • erythematous tender patches progressing to painful desquamation and superficial skin sloughing with “scalded” appearance
  • generalized and flaccid bullae
  • perioral and periorbital fissures
  • mucous membranes not involved
  • positive Nikolsky sign which describes a
    • separation of epidermis from dermis with slight pressure
  • distribution is often in the face, neck, groin, axillae, and other flexural surfaces
• mucosal surfaces not involved (vs. SJS & TEN)

Studies

• Labs
  • blood cultures typically negative
  • superficial wound cultures and bullae fluid cultures are sterile
• Biopsy
  • indications
    • if diagnosis of toxic epidermal necrolysis needs to be ruled out
• Histology
• intraepidermal cleavage

Differential

• Toxic epidermal necrolysis 
  • full thickness epidermal cell necrosis and supepidermal cleavage
• Bullous impetigo 
  • honey-crusted erosions
  • more widespread
• positive superficial wound cultures

Treatment

• Medical
  • intravenous anti-staphylococcal antibiotics
    • indications
      • administered in the treatment of staphlococcal scalded skin syndrome
    • modalities
      • nafcillin or oxacillin in methicillin-sesitive Staphylococcus aureus (MSSA)
• vancomycin in cases of methicillin-resistant Staphylococcus aureus (MRSA)

Complications

• Secondary infection of denuded skin