Staphylococcal scalded skin syndrome

Staphylococcal scalded skin syndrome (SSSS) is an acute skin condition caused by exfoliative toxins from Staphylococcus aureus. SSSS primarily affects infants and young children and most often follows a staphylococcal infection (e.g., pharyngitis, bullous impetigo), though the preceding infection may go unnoticed. Symptoms include skin tenderness, erythema, and fever, followed 1–2 days later by flaccid blisters and superficial skin sloughing that reveals moist red tissue (scalded appearance). Mucous membranes are spared. Diagnosis is clinical; biopsy is performed in the case of diagnostic uncertainty and reveals intraepidermal cleavage beneath the stratum corneum. Treatment involves antibiotics. SSSS generally heals without scarring.

• Rare condition (∼ 8 per 1,000,000 children) ^[1]
• Primarily affects infants and young children between 6 months and 5 years of age (peak incidence: 2–3 years) ^[2]
• Rare in adults: may occur in adults with predisposing conditions (e.g., impaired renal function or immunosuppression) ^[3]

Epidemiological data refers to the US, unless otherwise specified.

• Pathogen
  • Staphylococcus aureus strains that produce exfoliative toxins
  • Exfoliative toxin A and B have been shown to cleave desmoglein-1 in the granular layer of the epidermis → disruption of keratinocyte attachments ^[4]
• Route of infection: dissemination of toxins from a local infection
  • Following a staphylococcal infection elsewhere (e.g., skin, mouth, nose, throat, GI tract, umbilicus). The initial infection may also be completely undetected.
  • Following bullous impetigo

SSSS belongs to the spectrum of diseases mediated by specific staphylococcal toxins, which also includes bullous impetigo, toxic shock syndrome (TSS), and Staphylococcus aureus food poisoning. Unlike TSS, SSSS does not have systemic manifestations (involvement of, e.g., liver, kidney, bone marrow, CNS).

• Prodrome: fever, skin tenderness, irritability ^[5]
• Cutaneous lesions ^[5]
  • Initial manifestation
    • Erythematous macules localized to face, neck, and intertriginous areas
    • Perioral and periocular crusting
  • Progression over 48 hours
    • Flaccid, easily ruptured bullae
    • Positive Nikolsky sign
    • Widespread desquamation with moist red skin beneath (i.e., a “scalded” appearance)
    • Crusting and/or fissuring of the skin
• Systemic illness: : signs of hypovolemia (e.g., tachycardia, hypotension), hypo- or hyperthermia
• Symptoms from the primary site of S. aureus infection, e.g.:
  • Bacterial conjunctivitis
  • Bacterial sinusitis
  • Impetigo
  • Pharyngitis

Unlike Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), SSSS spares mucosal membranes. ^[5]

SSSS is a clinical diagnosis, typically supported by a history of a localized S. aureus infection and characteristic physical findings. Laboratory studies are performed in the case of diagnostic uncertainty. ^[5]

• Laboratory studies ^[5]
  • CBC: normal or ↑ WBCs
  • Bacterial cultures of potential sites of preceding S. aureus infection, e.g., blood, urine, nasopharynx, conjunctiva
• Skin biopsy (confirmatory study)
  • Indication: diagnostic uncertainty, e.g., to rule out SJS or TEN
  • Findings ^[3]
    • Superficial intraepidermal cleavage beneath the stratum corneum
    • No inflammatory cell infiltrate
    • No keratinocyte necrosis

Severe exfoliative skin conditions

Other exfoliative and blistering skin conditions ^[5]

• See “Overview of blistering skin diseases” and “Rash.”
• Kawasaki disease
• Toxic shock syndrome
• Pemphigus vulgaris
• Erythema multiforme
• Second-degree burns
• Graft-versus-host disease
• Sunburn

The differential diagnoses listed here are not exhaustive.

Antibiotic therapy ^[7][8]

• Patients without MRSA risk factors
  • Nafcillin (off-label) ^[7]
  • OR oxacillin (off-label) ^[7]
• Patients with MRSA risk factors: Vancomycin (off-label) ^[7]

Supportive care ^[5]

• Goal-directed IV fluid therapy: See “Strategies for IV fluid therapy.”
• Wound care, e.g., with nonadherent dressings and/or emollients
• Antipyretic therapy, e.g., acetaminophen
• Antipruritic therapy, e.g., cetirizine, diphenhydramine
• Pain management: See “Oral analgesics” and “Parenteral analgesics.”

Avoid NSAIDs, as they may decrease renal excretion of exotoxins. ^[5]

Steroids are contraindicated, as the etiology of SSSS is infectious. ^[8]

Disposition and monitoring ^[9]

• Admission is typically required; consider transfer to a burn center for severe disease.
• Monitor for acute complications, including:
  • Superinfection, septic shock
  • Hypovolemic shock
  • Hypothermia
• Treat complications, e.g., with:
  • Electrolyte repletion
  • Rewarming techniques
  • Broad spectrum antibiotics for sepsis

• Clinical diagnosis
• Skin biopsy if there is diagnostic uncertainty
• Antibiotic therapy
  • No MRSA risk factors: nafcillin or oxacillin
  • MRSA risk factors: vancomycin
• Goal-directed IV fluid therapy
• Wound care, e.g., with nonadherent dressings and/or emollients
• Antipyretic therapy
• Antipruritic therapy
• Pain management (avoid NSAIDs)
• Inpatient admission; burn center for severe disease

The complications faced by SSSS patients are similar to those of patients with burns, as both have a compromised skin barrier:

• Fluid and electrolyte imbalances
• Thermal dysregulation
• Secondary infections (e.g., pneumonia, sepsis)

We list the most important complications. The selection is not exhaustive.

• Mortality rate ^[10]
  • In children: < 5%
  • In adults: > 60%
• Blisters heal completely, without scarring, as skin cleavage is intraepidermal.