Staphylococcal-scalded skin syndrome: evaluation, diagnosis, and management

World J Pediatr. 2018 Apr;14(2):116-120. doi: 10.1007/s12519-018-0150-x. Epub 2018 Mar 5.

Abstract

Background: Staphylococcal-scalded skin syndrome (SSSS), also known as Ritter disease, is a potentially life-threatening disorder and a pediatric emergency. Early diagnosis and treatment is imperative to reduce the morbidity and mortality of this condition. The purpose of this article is to familiarize physicians with the evaluation, diagnosis, and treatment of SSSS.

Data sources: A PubMed search was completed in Clinical Queries using the key terms "Staphylococcal scalded skin syndrome" and "Ritter disease".

Results: SSSS is caused by toxigenic strains of Staphylococcus aureus. Hydrolysis of the amino-terminal extracellular domain of desmoglein 1 by staphylococcal exfoliative toxins results in disruption of keratinocytes adhesion and cleavage within the stratum granulosum which leads to bulla formation. The diagnosis is mainly clinical, based on the findings of tender erythroderma, bullae, and desquamation with a scalded appearance especially in friction zones, periorificial scabs/crusting, positive Nikolsky sign, and absence of mucosal involvement. Prompt empiric treatment with intravenous anti-staphylococcal antibiotic such as nafcillin, oxacillin, or flucloxacillin is essential until cultures are available to guide therapy. Clarithromycin or cefuroxime may be used should the patient have penicillin allergy. If the patient is not improving, critically ill, or in communities where the prevalence of methicillin-resistant S. aureus is high, vancomycin should be used.

Conclusion: A high index of suspicion is essential for an accurate diagnosis to be made and treatment promptly initiated.

Keywords: Blisters; Desquamation; Erythroderma; Exfoliative toxins; Staphylococcus aureus.

Publication types

  • Review

MeSH terms

  • Age Distribution
  • Anti-Bacterial Agents / administration & dosage*
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Methicillin-Resistant Staphylococcus aureus / drug effects
  • Methicillin-Resistant Staphylococcus aureus / pathogenicity*
  • Prognosis
  • Risk Assessment
  • Severity of Illness Index
  • Sex Distribution
  • Staphylococcal Scalded Skin Syndrome / diagnosis*
  • Staphylococcal Scalded Skin Syndrome / drug therapy*
  • Staphylococcal Scalded Skin Syndrome / epidemiology
  • Staphylococcus aureus / drug effects
  • Staphylococcus aureus / pathogenicity*
  • Treatment Outcome

Substances

  • Anti-Bacterial Agents