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)
    1. Age <1 year Incidence 25 cases per 100,000 children
    2. Overall Incidence rare: 0.56 per million people

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, Umbilicus, face)
      2. Non-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
  3. Young children are most susceptible
    1. Lack protective enzymes against staphylococcal toxin
    2. Immature Kidneys are less able to excrete exfoliative toxin

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. Decreased feeding
    5. Red, painful skin (tender to touch)

V. Signs

  1. Red, scarlatiniform, sandpaper-like rash
    1. Paper-thin skin that desquamates (Exfoliative Dermatitis)
    2. Distribution especially in the skin folds and flexor creases, perioral area, neck, axilla and groin
    3. Tender erythema
  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 in adults
    2. Children, by contrast typically have negative Blood Cultures
      1. Gray (2025) Pediatr Dermatol 42(5):978-84 +PMID: 40650480 [PubMed]
  2. Skin biopsy
  3. Exotoxin assay
  4. Avoid skin lesion cultures
    1. Lesions are typically sterile and will be non-diagnostic

VII. Differential Diagnosis

  1. See Bullous Conditions
  2. Life Threatening and other severe causes
    1. Steven Johnson Syndrome
    2. Toxic Epidermal Necrolysis
    3. Scarlet Fever
    4. Multisystem Inflammatory Syndrome in Children (MIS-C, Kawasaki Disease)
    5. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS Syndrome)
  3. Other causes
    1. Candidiasis
    2. Impetigo
    3. Cellulitis
    4. Atopic Dermatitis
    5. Seborrheic Dermatitis

VIII. Labs

  1. Indicated in most suspected cases (unless isolated rash in a well appearing, hemodynamically stable infant)
  2. Complete Blood Count (CBC)
  3. Serum Electrolyte panel (basic chemistry panel, chem8)
  4. Urinalysis
  5. Blood Cultures
  6. Skin Cultures
  7. Consider Nasal swabs
    1. MRSA nasal swab in patient
    2. Staphyococcus aureus toxin swab of Caregivers (treat positive carriers with oral Antibiotics)
  8. Skin biopsy
    1. Consider if concerns for alternative diagnosis (e.g. Toxic Epidermal Necrolysis)

IX. Imaging

  1. Chest XRay
    1. If suspected lower respiratory source

X. Management

  1. 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)
    2. Topical Antibiotics are insufficient alone (exotoxin spreads systemically)
  2. Supportive Care
    1. Intravenous Fluids
    2. Local wound care (skin Emollients, nonadherent dressing)
    3. Analgesics
    4. Systemic Corticosteroids may be used in non-toxic appearing patients
  3. Adjunctive measures in severe cases
    1. Intravenous Immunoglobulin
    2. Plasmapheresis
  4. Disposition
    1. Admit patients with diffuse involvement to ICU or burn unit
    2. Mild cases may be considered for outpatient management on oral Antibiotics with close interval follow-up
      1. Localized rash
      2. No systemic symptoms or signs
      3. Maintain adequate hydration

XI. Course

  1. Symptoms start to improve at 1-2 days after treatment started
  2. Resolution in 5-7 days after Antibiotics are initiated
  3. Dry, peeling skin may persist for up to 1 week

XII. Prognosis

  1. Early diagnosis and treatment is associated with good prognosis
  2. Mortality in children overall: 5%

XIII. Resources

  1. Staphylococcal Scalded Skin Syndrome (Rare Disease Database, accessed 1/13/2022)
    1. https://rarediseases.org/rare-diseases/staphylococcal-scalded-skin-syndrome/

XIV. References

  1. Andriescu, Constien and Hill (2026) Crit Dec Emerg Med 40(4): 15-6
  2. Long (2016) Crit Dec Emerg Med 30(7):3-10
  3. Stewart (2022) Crit Dec Emerg Med 36(1): 16-7

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