Staphylococcal scalded skin syndrome (SSSS)

- is a syndrome of acute exfoliation of the skin following an erythematous cellulitis.

- SSSS also is known as Ritter von Ritterschein disease in newborns, Ritter disease, Lyell disease, and staphylococcal epidermal necrolysis.

- Pathophysiology:  A toxin produced by phage group 2 Staphylococcus aureus causes SSSS. An infection commonly occurs at a site such as the oral or nasal cavities, throat, or umbilicus. Two epidermolytic toxins (A and B) are then produced and act at a remote site leading to a red rash and separation of the epidermis beneath the granular cell layer. This is due to binding of the toxins to desmoglein 1 in desmosomes, causing it to break down. Bullae form, and diffuse sheetlike desquamation occurs. Involvement may occasionally be localized or patchy rather than diffuse. Healing typically occurs within 1-2 weeks.

- occurs mostly in children younger than 5 years, particularly neonates because 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 make neonates the most at risk. 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 fluid-filled blisters form. These rupture easily, leaving an area that looks like a burn.

- Diagnosis of SSSS is often suspected from the characteristic history and physical examination.

- The diagnosis may be confirmed with a biopsy and bacterial culture.

- Treatment 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. 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.

-  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.