Staphylococcal scalded skin syndrome
What is staphylococcal scalded skin syndrome?
Staphylococcal scalded skin syndrome (SSSS) is an illness characterised by red blistering skin that looks like a burn or scald, hence its name staphylococcal scalded skin syndrome. SSSS is caused by the release of two exotoxins (epidermolytic toxins A and B) from toxigenic strains of the bacteria Staphylococcus aureus. Desmosomes are the part of the skin cell responsible for adhering to the adjacent skin cell. The toxins bind to a molecule within the desmosome called Desmoglein 1 and break it up so the skin cells become unstuck.
SSSS has also been called Ritter disease or Lyell disease when it appears in newborns or young infants.
Who is at risk of staphylococcal scalded skin syndrome?
SSSS occurs mostly in children younger than 5 years, particularly neonates (newborn babies). Lifelong protective antibodies against staphylococcal exotoxins are usually acquired during childhood which makes SSSS much less common in older children and adults. Lack of specific immunity to the toxins and an immature renal clearance system (toxins are primarily cleared from the body through the kidneys) make neonates the most at risk.
Immunocompromised individuals and individuals with renal failure, regardless of age, may also be at risk of SSSS.
How do you get staphylococcal scalded skin syndrome?
SSSS starts from a localised staphylococcal infection that is a producer of the two causative exotoxins (epidermolytic toxins A and B). Outbreaks of SSSS often occur in childcare facilities. An asymptomatic adult carrier of Staphylococcus aureus introduces the bacteria into the nursery. About 15-40% of healthy humans are carriers of Staphylococcus aureus, that is, they have the bacteria on their skin without any sign of infection or disease (colonisation). However, staphylococcal skin infections are seen commonly in infants and younger children, thus an obvious increased risk of SSSS. Staphylococcus aureus is also commonly found in infections of the throat, ears and eyes.
What are the signs and symptoms of staphylococcal scalded skin syndrome?
SSSS usually starts with fever, irritability and widespread redness of the skin. Within 24-48 hours fluid-filled blisters form. These rupture easily, leaving an area that looks like a burn.
Characteristics of the SSSS rash include:
- Tissue paper-like wrinkling of the skin is followed by the appearance of large fluid-filled blisters (bullae) in the armpits, groin and body orifices such as the nose and ears.
- Rash spreads to other parts of the body including the arms, legs and trunk. In newborns, lesions are often found in the diaper area or around the umbilical cord.
- Top layer of skin begins peeling off in sheets, leaving exposed a moist, red and tender area. Nikolsky sign is positive (ie gentle strokes result in exfoliation)
Other symptoms may include tender and painful areas around the infection site, weakness, and dehydration.
How is staphylococcal scalded skin syndrome fever diagnosed?
Diagnosis of SSSS depends on:
- History and physical examination
- Tzanck smear
- Skin biopsy, which shows intraepidermal cleavage at the granular layer
- Bacterial culture from skin, blood, urine or umblical cord sample (in a newborn baby)
What is the treatment of staphylococcal scalded skin syndrome?
Treatment of SSSS usually requires hospitalisation, as intravenous antibiotics are generally necessary to eradicate the staphylococcal infection. A penicillinase-resistant, anti-staphylococcal antibiotic such as flucloxacillin is used. Other antibiotics include nafcillin, oxacillin, cephalosporin and clindamycin. Vancomycin is used in infections suspected with methicillin resistance (MRSA). Depending on response to treatment, oral antibiotics can be substituted within several days. The patient may be discharged from hospital to continue treatment at home.
Corticosteroids slow down healing and hence are not given to patients with SSSS.
Other supportive treatments for SSSS include:
- Paracetamol when necessary for fever and pain.
- Monitoring and maintaining fluid and electrolyte intake.
- Skin care (the skin is often very fragile). Petroleum jelly should be applied to keep the skin moisturised.
- Newborn babies affected by SSSS are usually kept in incubators.
Although the outward signs of SSSS look bad, children generally recover well and healing is usually complete within 5–7 days of starting treatment.
What are the complications from staphylococcal scalded skin syndrome?
SSSS usually follows a benign course when diagnosed and treated appropriately. However, if left untreated or treatment is unsuccessful, severe infections such as sepsis, cellulitis, and pneumonia may develop. Death can follow severe infection.
How to prevent staphylococcal scalded skin syndrome
If there is an outbreak of SSSS in either a neonatal care unit or childcare facility, the possibility of a staphylococcal carrier in the vicinity should be investigated. Identification of the healthcare worker, childcare worker, parent or visitor colonised or infected with Staphylococcus aureus is key to managing the problem. Once identified these individuals should be treated with oral antibiotics to eradicate the causative organism. To prevent further infections these places should employ strict hand washing with antibacterial soap or sanitisers.
- Staphylococcal Scalded Skin Syndrome – NORD National Organization for Rare Disorders. Accessed January 2016
- The management of staphylococcal scalded skin syndrome in infants – Nursing Times. Accessed January 2016
- Staphylococcal Scalded Skin Syndrome (SSSS) in Children – Johns Hopkins Medicine. Accessed January 2016
On DermNet NZ:
- Dermatologic Manifestations of Staphylococcal scalded skin syndrome – Medscape Reference
- Staphylococcal Scalded Skin Syndrome – British Association of Dermatologists
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