Staphylococcal Scalded Skin Syndrome


Staphylococcal Scalded Skin Syndrome, Staphylococcus Scalded Skin Syndrome, Ritters Disease

  • Epidemiology
  1. Typically affects age <2 years old
  • Pathophysiology
  1. Hematologic dissemination of Staphylococcus aureus from an infectious source (e.g. Otitis Media, URI)
    1. Contrast with Bullous Impetigo, where localized infection spreads contiguously
  2. Staphylococcus aureus strains that carry exfoliative toxins A and B (only 5% of S. aureus strains)
    1. Breaks down desmoglein-1 resulting in Acantholysis (breakage of cell to cell adhesions)
    2. Similar to Pemphigus Vulgaris (which in contrast is immune mediated)
  • Symptoms
  1. Prodrome (initial Upper Respiratory Infection)
    1. Otitis Media
    2. Pharyngitis
    3. Conjunctivitis
  2. Acute phase (initial, prior to Desquamation)
    1. Fever
    2. Malaise
    3. Red, scarlatiniform rash
    4. Red, painful skin
  • Signs
  1. Paper-thin skin that desquamates
  2. Large, flaccid Blisters, especially in the flexor creases
  3. No mucous membrane involvement
  4. Positive Nikolsky Sign (on affected skin)
  • Labs
  1. Blood Cultures
    1. Bacteremia is often present in Staphylococcal Scalded Skin Syndrome
  2. Skin biopsy
  3. Exotoxin assay
  4. Avoid skin lesion cultures
    1. Lesions are sterile and will be non-diagnostic
  • Management
  1. Admit patients with diffuse involvement to ICU or burn unit
  2. Intravenous antibiotics covering Staphylococcus aureus (consider MRSA coverage depending on risk)
  3. Systemic Corticosteroids may be used in non-toxic appearing patients
  4. Adjunctive measures in severe cases
    1. Intravenous Immunoglobulin
    2. Plasmapheresis
  • Differential Diagnosis
  1. See Bullous Conditions
  2. Steven Johnson Syndrome
  3. Toxic Epidermal Necrolysis
  • References
  1. Long (2016) Crit Dec Emerg Med 30(7):3-10