Staphylococcal Scalded Skin Syndrome

Basics

DESCRIPTION

  • A spectrum of generalized exfoliative skin disease with blistering of the upper layer of skin caused by an epidermolytic toxin produced by certain strains of Staphylococcus aureus
  • In neonates and young infants, also known as Ritter disease or pemphigus neonatorum
  • Classically described as skin tenderness and erythema, with bullae formation and desquamation
  • Severity of the disease ranges from
    • Few blisters localized to site of infection
    • Mild illness with desquamation of skinfolds following impetigo
    • Generalized severe exfoliation involving much of the body (typically seen in neonates)
    • Classic staphylococcal scalded skin syndrome (SSSS): tenderness, erythema, desquamation, or bullae formation; may resemble scalding injury

EPIDEMIOLOGY

  • Most cases occur in neonates and children <5 years of age, although can occur at any age.
  • Rare in adults due to increased circulating antibodies and adult kidney excretion of the toxin

INCIDENCE

  • No differences in incidence based on gender in children; however, in adults, the male-to-female ratio is 2:1.
  • Increased incidence in children reported during summer and fall months

RISK-FACTORS

  • Immunocompromised state (in children or adults)
    • Maternal antibodies transferred via breast milk are partially protective, but neonatal cases can still occur.
  • Increased S. aureus carriage and susceptibility to toxin (usually in adults)
  • Renal impairment either due to immature renal clearance of toxin in children or underlying renal disease

GENERAL-PREVENTION

  • Good hand hygiene practices, including adherence to contact precautions in hospitalized patients, to prevent spread from asymptomatic carriers
  • Prevent skin from becoming overly moist or macerated.
  • Isolation of hospitalized patient
    • Suspected or documented cases should be placed in contact isolation.

PATHOPHYSIOLOGY

  • Results from S. aureus infection and subsequent exfoliative toxin production
  • Exfoliative toxins circulate throughout the body, causing blisters at sites distant from the infection.
  • Destruction of protein desmoglein 1 (attachment protein found only in the superficial epidermis) by exfoliative toxin A (ETA) and exfoliative toxin B (ETB) causes intraepidermal splitting leading to bullae development and skin desquamation.

ETIOLOGY

Exfoliative toxin released by S. aureus:

  • Two major serotypes of the toxin: ETA and ETB
  • Mostly caused by S. aureus belonging to phage group II, types 71 and 55

ASSOCIATED-CONDITIONS

  • Normal skin colonization with S. aureus with break in skin leading to systemic infection
  • Skin and soft tissue infections or abscesses
  • Bullous impetigo

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