Staphylococcal Scalded Skin Syndrome

Aka: Staphylococcal Scalded Skin Syndrome, Staphylococcal Scalded Skin Infection, Staphylococcus Scalded Skin Syndrome, Ritters Disease

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II. Epidemiology

  1. Typically affects age <5 years old (and especially <2 years)
  2. Incidence: <0.56 per Million (rare)

III. Pathophysiology

  1. Group 2 Coagulase Positive Staphylococcus aureus strains
    1. Strains that carry exfoliative toxins A and B (only 5% of S. aureus strains)
    2. Breaks down desmoglein-1 resulting in epidermolysis or Acantholysis (breakage of cell to cell adhesions)
    3. Similar to Pemphigus Vulgaris (which in contrast is immune mediated)
  2. Sources
    1. Initial localized infection
      1. Skin sites (e.g. Diaper Dermatitis, ubilicus, face)
      2. Nn-skin sites (e.g. Otitis Media, Upper Respiratory Infection
    2. Later, hematologic dissemination of Staphylococcus aureus from an infectious source
      1. Contrast with Impetigo which spreads contiguously

IV. 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. Irritability
    4. Red, painful skin

V. Signs

  1. Red, scarlatiniform, sandpaper-like rash
    1. Paper-thin skin that desquamates (Exfoliative Dermatitis)
    2. Distribution especially in the flexor creases, perioral area, neck and groin
  2. Large, flaccid Blisters
    1. Positive Nikolsky Sign (on affected skin)
  3. No mucous membrane involvement

VI. 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

VII. Differential Diagnosis

  1. See Bullous Conditions
  2. Steven Johnson Syndrome
  3. Toxic Epidermal Necrolysis
  4. Scarlet Fever
  5. Multisystem Inflammatory Syndrome in Children (MIS-C, Kawasaki Disease)
  6. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS Syndrome)

VIII. Management

  1. Admit patients with diffuse involvement to ICU or burn unit
  2. Intravenous Antibiotics covering Staphylococcus aureus (consider MRSA coverage depending on risk)
    1. Methicillin Sensitive Staphylococcus aureus infections account for 95% of cases (as of 2018)
  3. Systemic Corticosteroids may be used in non-toxic appearing patients
  4. Adjunctive measures in severe cases
    1. Intravenous Immunoglobulin
    2. Plasmapheresis

IX. Course

  1. Resolution in 5-7 days after Antibiotics are initiated

X. References

  1. Long (2016) Crit Dec Emerg Med 30(7):3-10
  2. Stewart (2022) Crit Dec Emerg Med 36(1): 16-7
  3. Rare Disease Database, accessed 1/13/2022
    1. https://rarediseases.org/rare-diseases/staphylococcal-scalded-skin-syndrome/

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