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SSSS is caused by the release of exotoxins (epidermolytic toxins A and B) from toxigenic strains of 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 usually occurs in children < 5 years, particularly neonates. Immunocompromised individuals and individuals with renal failure, regardless of age, may also be at risk of SSSS.
SSSS usually starts with fever, irritability and widespread redness of the skin. Within 24 – 48 hours tissue paper-like wrinkling of the skin is followed by the formation of fluid-fllled blisters in the armpits, groin and body oriflces such as the nose and ears. These rupture easily, leaving an area that looks like a burn. Nikolsky sign is positive (i.e. gentle strokes result in exfoliation). The 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.
Diagnosis of SSS is with history and examination, Tzanck smear, skin biopsy and bacterial culture. The main differential is SJS/TENS; SSS, in contrast to toxic epidermal necrolysis, does not affect the oral mucosa and this may be a helpful clue to diagnosis.
Treatment of SSSS usually requires hospitalisation, as intravenous antibiotics (e.g. flucloxacillin) are generally necessary to treat the infection. Supportive treatment includes paracetamol for pain and fever, monitoring and maintenance of fluid and electrolytes, skin care and temperature regulation. 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. Although the outward signs of SSSS look bad, children generally recover well and healing is usually complete within 5 – 7 days of starting treatment. If SSSS is not recognised and treated early, complications include sepsis, secondary cellulitis, and pneumonia may occur.