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
Commonly 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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Citation
Cabana, Michael D., editor. "Staphylococcal Scalded Skin Syndrome." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617332/2.1/Staphylococcal_Scalded_Skin_Syndrome.
Staphylococcal Scalded Skin Syndrome. In: Cabana MDM, ed. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617332/2.1/Staphylococcal_Scalded_Skin_Syndrome. Accessed December 1, 2024.
Staphylococcal Scalded Skin Syndrome. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult (8th ed.). Wolters Kluwer. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617332/2.1/Staphylococcal_Scalded_Skin_Syndrome
Staphylococcal Scalded Skin Syndrome [Internet]. In: Cabana MDM, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2024 December 01]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617332/2.1/Staphylococcal_Scalded_Skin_Syndrome.
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