Staphylococcal scalded Skin Syn-drome (SSSS)

Notes

## Basic introduction

  • SSSS is a toxin-mediated epidermolytic condition that leads to detachment of superficial epidermal layers, leading a scalded skin appearance.
  • SSSS is also known as Ritter disease or Lyell disease (especially when it appears in newborns or young infants).

## Pathogenesis of SSSS

  • Toxigenic strains of Staphylococcus aureus releases of two exotoxins - epidermolytic toxins A and epidermolytic toxins B (which are serine proteases)
  • The toxins bind to Desmoglein 1 within the desmosome and break it up making the granulosum and spinosum to be separated. (Desmosome is the part of the skin cell responsible for adhering to the adjacent skin cell).
  • It is thought that epidermolytic toxins in the body are cleared efficiently in immunocompetent persons but poorly so in immunocompromised persons and children (as their immune system is not fully developed)
  • The complications of SSSS include dehydration, cellulitis, sepsis and pneumonia

##Statistics

  • The global incidence of SSSS is estimated to be 0.09 - 0.13 per million people.
  • SSSS mainly affects neonates and children < 6 years (about 98% of all cases while 62% of all cases affects children below 2 years), though it can affect adults, especially those who are immunocompromised or are suffering from renal failure.
  • The mortality rate in infants is about 4%, though it can as high as 67% in adults.
Symptoms
  • Fever
  • Generalized erythema
  • Skin tenderness (sometimes)
  • Sore throat (sometimes)
  • Conjunctivitis
  • Superficial blisters (flaccid bullae)
  • Nikolsky sign-positive test (soft shearing force on skin causes the upper epidermis to slip)
  • Widespread blisters on the skin (sometimes)
Diagnosis
  • Clinical review
  • Swab, culture and sensitivity
Differential
  • Bullous impetigo
  • Pemphigus
  • Scarlet fever
  • Toxic epidermal necrolysis (TEN)
  • Toxic shock syndrome
Prevention
  • Testing for Staphylococcus aureus in the family of the patient
Reference

1. Conway DG, Lyon RF, Heiner JD; A desquamating rash; staphylococcal scalded skin syndrome. Ann Emerg Med. 2013 Jan; 61(1):118, 129.

2. Kenyan Ministry of Health. Clinical guidelines for management and referral of common conditions at levels 4-6. Hospitals. 2009; 3:259-261.http://apps.who.int/medicinedocs/documents/s21000en/s21000en.pdf

3. Ministry of Health, Kenya. Kenya Essential Medicine List (2016). http://publications.universalhealth2030.org/uploads/KEML-2016Final-1.pdf

4. Oliveira AR, Aires S, Faria C, et al; Staphylococcal scalded skin syndrome. BMJ Case Rep. 2013 Jun 10;2013

5. Patel NN, Patel DN; Staphylococcal scalded skin syndrome. Am J Med. 2010 Jun; 123(6):505-7.

Management
  • Handle like burn patient
  • Barrier nursing may be required
  • Maintain fluids and electrolyte balance
  • Antibiotic therapy with oral or parenteral cloxacillin or flucloxacillin or an antibiotic that has been determined by culture and sensitivity results
  • Topical care bath with normal saline
  • Mupirocin or fusidic acid topical preparations can be used
  • Analgesics, starting with paracetamol
  • Physiotherapy
  • Adequate nutrition
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