Staphylococcal Scalded Skin Syndrome

Background:

• Characterized by skin exfoliation, or sloughing of the outer layers of the epidermis
• Caused by exfoliative toxin A or B, which is secreted by Staphylococcus aureus

Epidemiology:

• Predominately affects neonates and infants
• 3-4% of methacillin-sensitive Staphylococcus aureus (MSSA) strains carry the gene for exfoliative toxin A or B
• 10% of methacillin-resistant Staphylococcus aureus (MRSA) carry the exfoliative toxin A gene

Mechanism:

• Exfoliative toxins are serine proteases that specifically target desmoglein 1, a cadherin adhesion protein in the desmosomes of the stratum granulosum of the epidermis
  • Cleavage of desmoglein I severs the intracellular adhesion between keratinocytes in the stratum granulosum, resulting in separation between the stratum granulosum and the stratum corneum

Clinical Symptoms:

• Fever, malaise, lethargy, and poor feeding followed by erythematous rash with large fluid-filled blisters
  • These blisters burst easily with mechanical abrasion (positive Nikolsky sign)
  • Areas affected: neck folds, diaper area, axilla

Diagnosis:

• Diagnosis is usually clinical
• Culture nose, throat, and other possible foci for infection based on clinical presentation
• PCR for exfoliative toxin-encoding gene
• WBC usually normal to elevated, ESR often elevated, blood culture usually negative,
  • Do not culture from exfoliated regions, these are sterile
• Chest X-ray if pneumonia is suspected

Differential Diagnosis:

• Toxic Epidermal Necrolysis (TEN) or Stevens Johnson Syndrome (SJS):
  • Separation at the dermal-epidermal junction and classically involves the mucosal surfaces (mouth, esophagus, conjunctiva, anus, vagina), while SSSS causes separation more superficially within the stratum granulosum and spares the mucosa
• Bullous impetigo:
  • These lesions are smaller and occur only where there is local Staphlococcus aureus infection
  • Both conditions are caused by exfoliative toxin, but differ in the extent of skin damage
• Epidermolysis bullosa:
  • Congenital defect involving dermal-epidermal junction, leading to skin fragility
• Thermal or chemical burns

Treatment:

• IV antibiotics to cover S. aureus
  • Penicillinase-resistant penicillins such as nafcillin or oxacillin until clinical improvement
    • Most strains associated with SSSS produce penicillinase
    • Transition to oral antibiotics and continue for an additional 5-8 days
    • Antibiotic choice and duration of treatment depends largely on original focus of infection
  • If MRSA is suspected, coverage is indicated (vancomycin or linezolid)
  • Clindamycin often used as adjuvant therapy due to its inhibition of exfoliative toxin production (inhibits 50S ribosomal subunit)
• Supportive care
  • Adequate hydration to offset increased insensible fluid loss from denuded skin
  • Apply hydrating ointment or cream and moist compresses to affected areas as a barrier to prevent fluid loss as well as secondary infection
  • Antipyretics (ibuprofen, aspirin, acetaminophen)

​Prognosis:

• Complete resolution within 10 days of treatment
• Mortality among treated children 5%

Helpful websites for families:

http://www.dermnetnz.org/topics/staphylococcal-scalded-skin-syndrome/

http://emedicine.medscape.com/article/788199-overview

https://rarediseases.org/rare-diseases/staphylococcal-scalded-skin-syndrome/

References:

Bukowski, Michal, Benedykt Wladyka, and Grzegorz Dubin. “Exfoliative Toxins of Staphylococcus Aureus.” Toxins 2, no. 5 (May 25, 2010): 1148–65. doi:10.3390/toxins2051148.

Cribier, Bernard, Yves Piemont, and Edouard Grosshans. “Staphylococcal Scalded Skin Syndrome in Adults.” Journal of the American Academy of Dermatology 30, no. 2 (February 1, 1994): 319–24. doi:10.1016/S0190-9622(94)70032-X.

Gemmell, C. G. “Staphylococcal Scalded Skin Syndrome.” Journal of Medical Microbiology 43, no. 5 (November 1995): 318–27. doi:10.1099/00222615-43-5-318.

Johnson, W. M., S. D. Tyler, E. P. Ewan, F. E. Ashton, D. R. Pollard, and K. R. Rozee. “Detection of Genes for Enterotoxins, Exfoliative Toxins, and Toxic Shock Syndrome Toxin 1 in Staphylococcus Aureus by the Polymerase Chain Reaction.” Journal of Clinical Microbiology 29, no. 3 (March 1991): 426–30.

Ladhani, S., C. L. Joannou, D. P. Lochrie, R. W. Evans, and S. M. Poston. “Clinical, Microbial, and Biochemical Aspects of the Exfoliative Toxins Causing Staphylococcal Scalded-Skin Syndrome.” Clinical Microbiology Reviews 12, no. 2 (April 1999): 224–42.

Mégevand, C., A. Gervaix, U. Heininger, C. Berger, C. Aebi, B. Vaudaux, C. Kind, et al. “Molecular Epidemiology of the Nasal Colonization by Methicillin-Susceptible Staphylococcus Aureus in Swiss Children.” Clinical Microbiology and Infection: The Official Publication of the European Society of Clinical Microbiology and Infectious Diseases 16, no. 9 (September 2010): 1414–20. doi:10.1111/j.1469-0691.2009.03090.x.

Payne, Aimee S, Yasushi Hanakawa, Masayuki Amagai, and John R Stanley. “Desmosomes and Disease: Pemphigus and Bullous Impetigo.” Current Opinion in Cell Biology 16, no. 5 (October 2004): 536–43. doi:10.1016/j.ceb.2004.07.006.

Raymond, J., E. Bingen, N. Brahimi, M. Bergeret, J. Lepercq, J. Badoual, and D. Gendrel. “Staphylococcal Scalded Skin Syndrome in a Neonate.” European Journal of Clinical Microbiology & Infectious Diseases: Official Publication of the European Society of Clinical Microbiology 16, no. 6 (June 1997): 453–54.

Sakurai, S., H. Suzuki, and K. Machida. “Rapid Identification by Polymerase Chain Reaction of Staphylococcal Exfoliative Toxin Serotype A and B Genes.” Microbiology and Immunology 39, no. 6 (1995): 379–86.

Sila, Jaromir, Pavel Sauer, and Milan Kolar. “Comparison of the Prevalence of Genes Coding for Enterotoxins, Exfoliatins, Panton-Valentine Leukocidin and Tsst-1 between Methicillin-Resistant and Methicillin-Susceptible Isolates of Staphylococcus Aureus at the University Hospital in Olomouc.” Biomedical Papers of the Medical Faculty of the University Palacky, Olomouc, Czechoslovakia 153, no. 3 (September 2009): 215–18.

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