• Clinical science

Staphylococcal scalded skin syndrome (Ritter's disease…)

Abstract

Staphylococcal scalded skin syndrome (SSSS) is a potentially serious acute skin condition caused by the exfoliative toxins of Staphylococcus aureus and typically affects infants and young children. It is usually preceded by a mucocutaneous staphylococcal infection, such as pharyngitis or bullous impetigo, though this preceding infection may go unnoticed by patients and other caregivers. Following systemic dissemination of toxins from the local infection, SSSS itself typically begins with skin tenderness, erythema, and fever. This is followed a day or two later by flaccid blisters and sloughing off of the superficial layer of skin to reveal moist, red tissue underneath, giving the area a “scalded”-looking appearance. Mucous membranes are spared. A presumptive diagnosis of SSSS is based on clinical findings. Biopsy is only performed in unclear cases and shows separation of the epidermis at the granular layer. Treatment involves the administration of antibiotics and potential intensive care monitoring. The prognosis is generally good, and blisters heal without significant scarring.

Epidemiology

  • Rare condition
  • Primarily affects infants and young children; (98% of patients are < 6 years of age)
  • A few exceptional cases have been reported in adults with immunocompromise or chronic illness, however, SSSS remains very rare in adults.

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • Pathogen: Staphylococcus aureus strains that produce exfoliative toxins
  • :
  • Route of infection: dissemination of toxins from a local infection

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 (e.g., liver, kidney, bone marrow, and CNS involvement)!

References:[2][3][4][1]

Clinical features

Initially

  • Fever, malaise, and irritability
  • Skin tenderness
  • Diffuse or localized erythema, often beginning periorally
    • Localization
      • Infants: perianal, perineal, periumbilical
      • Children: extremities
      • Very ill children: generalized lesions

After 24–48 hours

  • Flaccid, easily ruptured blisters that break to reveal moist, red skin beneath (i.e., with a “scalded” appearance) → widespread sloughing of epidermal skin
  • Nikolsky's sign
  • No mucosal involvement
  • Cracking, and crusting is common
  • Signs of shock (hypotension, tachycardia)

Stevens-Johnson syndrome and toxic epidermal necrolysis present with mucosal involvement, SSSS doesn't!

References:[1][5]

Diagnostics

The presumptive diagnosis of SSSS is made based on clinical findings. Cultures (e.g., blood or nasopharynx) are usually taken for confirming the diagnosis, and a biopsy may be performed to exclude suspected differential diagnoses, but is usually not required.

  • History: localized staphylococcal infection; (e.g., pharyngitis, bullous impetigo)
  • Laboratory tests: for confirming the diagnosis
    • WBCs
    • ESR
    • Cultures of potential sites of preceding infection (blood, urine, abnormal skin, nasopharynx, umbilicus, or any other suspected focus)
  • Biopsy: indicated in unclear cases, especially when TEN or SJS are suspected
    • Intraepidermal fissure and blister formation at the granular layer
    • Lack of inflammatory cell infiltrate
  • Imaging
    • Chest x-ray: to rule out pneumonia as an original source of infection

References:[1][6][7]

Differential diagnoses

Differential diagnoses of severe exfoliative skin conditions
SSSS SJS TEN
Age of typical patient
  • Children < 6 years
  • Adults
  • Adults
Etiology
  • Adverse drug reaction (Infectious etiologies exist but are less common)
  • Adverse drug reaction (Infectious etiologies exist but are less common)
Clinical features
  • Sloughing of skin, Nikolsky's sign
  • Mucous membrane involvement
  • Typically < 10% of total body surface area
  • Sloughing of skin, Nikolsky's sign
  • ≥ 2 mucous membranes involved
  • > 30% of total body surface area
Biopsy

References:[3][8]

The differential diagnoses listed here are not exhaustive.

Treatment

  • Hospital admission
    • Necessary in most cases
    • Isolation recommended
    • Severe cases may be treated in the burn unit or intensive care unit.
    • Outpatient treatment is possible in older children who are eating and drinking well and have minimal skin involvement.
  • IV antibiotics
  • Supportive care
    • Fluid rehydration as indicated
    • Supportive skin care: emollients; , covering denuded areas
    • NSAIDs as indicated for pain and fever

Steroids are contraindicated, as the etiology of SSSS is infectious! (They are, however, indicated in SJS and TEN.)

References:[1][5][7][9]

Complications

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)

References:[8]

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

Prognosis

References:[7][10]

  • 1. King RW. Staphylococcal Scalded Skin Syndrome. In: Staphylococcal Scalded Skin Syndrome. New York, NY: WebMD. http://emedicine.medscape.com/article/788199-overview. Updated June 15, 2016. Accessed May 4, 2017.
  • 2. Le T, Bhushan V, Chen V, King M. First Aid for the USMLE Step 2 CK. McGraw-Hill Education; 2015.
  • 3. Le T, Bhushan V, Sochat M, Petersen M, Micevic G, Kallianos K. First Aid for the USMLE Step 1 2014. McGraw-Hill Medical; 2014.
  • 4. Yamasaki O, Yamaguchi T, Sugai M. Clinical Manifestations of Staphylococcal Scalded-Skin Syndrome Depend on Serotypes of Exfoliative Toxins. J Clin Microbiol. 2005; 43(4): pp. 1890–1893. doi: 10.1128/JCM.43.4.1890-1893.2005.
  • 5. Garfunkel LC, Kaczorowski J, Christy C. Pediatric Clinical Advisor E-Book: Instant Diagnosis and Treatment. Elsevier Health Sciences; 2007.
  • 6. Ladhani S, Robbie S, Garratt RC, Chapple DS, Joannou CL, Evans RW. Development and Evaluation of Detection Systems for Staphylococcal Exfoliative Toxin A Responsible for Scalded-Skin Syndrome. J Clin Microbiol. 2001; 39(6): pp. 2050–2054. doi: 10.1128/JCM.39.6.2050-2054.2001.
  • 7. Pielop JA. Vesiculobullous and pustular lesions in the newborn. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/vesiculobullous-and-pustular-lesions-in-the-newborn. Last updated March 16, 2016. Accessed May 4, 2017.
  • 8. Le T, Bhushan V, Bagga HS. First Aid for the USMLE Step 2 CK. McGraw-Hill Medical; 2009.
  • 9. hopkinsmedicine.org. Staphylococcal Scalded Skin Syndrome (SSSS) in Children. http://www.hopkinsmedicine.org/healthlibrary/conditions/dermatology/staphylococcal_scalded_skin_syndrome_85,P00316/. Accessed May 4, 2017.
  • 10. Patel GK, Finlay AY. Staphylococcal scalded skin syndrome: diagnosis and management. Am J Clin Dermatol. 2003; 4(3): pp. 165–175. pmid: 12627992.
  • National Organization for Rare Disorders. Staphylococcal Scalded Skin Syndrome. https://rarediseases.org/rare-diseases/staphylococcal-scalded-skin-syndrome/. Accessed May 4, 2017.
last updated 10/19/2018
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