Staphylococcal scalded skin syndrome (SSSS)

Staphylococcal scalded skin syndrome is also known as Ritter disease, characterized by erythematous tender macule, which coalesce rapidly to cover large area , skin peels from the effected area in sheet and rarely forms blister due to collection of exudates beneath the involved skin. This disorder is caused by the exotoxin “exfoliative toxins”(ET) released from strains of Staphylococcus aureus.

Histologically there is a separation in the epidermis, just beneath the granular cell layer. Two types of staphylococcal scalded skin syndrome exist: a localized form, in which there is a limited involvement of the epidermis, and the generalized form, in which there is a large area involvement.

Two exfoliative toxins (ET-A and ET-B) are known to cause this syndrome, but the exact mechanism by which they cause exfoliation is not clear. However its believed that the toxins act as proteases, target the desmoglein-1, responsible for cell-to-cell adhesion in the epidermis. SSSS commonly seen in the infants and children because of the immature kidney in children that fails to clear the circulating exotoxins, in the adults it is encountered in persons with renal disease because of the same reason .Other postulated theory is exfoliative toxins possess a superantigenic property which is also is responsible for detachment of the skin.


Initial studies had suggested that phage l group II S aureus (subtypes 3A, 3B, 3C, 55 and 71) were solely responsible for exfoliative toxin production, but it is now known that all phage groups are capable of producing exfoliative toxin and can cause staphylococcal scalded skin syndrome.

Staphylococcal scalded skin syndrome differs from the toxic epidermal necrolysis (TEN), in the cleavage site, in staphylococcal scalded skin syndrome the cleavage is at the granular layer of epidermis, opposed to the TEN, where there is necrosis of the full thickness of epidermal layer. Mucosa is never involved in SSSS, where as mucosa is always involved in TEN. Treatment of SSSS is administration of appropriate antibiotic, where as treatment of TEN is stopping the offending agent, maintenance of nutrition and electrolyte imbalance, in early stage of TEN(drug induced) the suspected drug is stopped, and to arrest the progression of the TEN, suprapharmacological dose of corticosteroid may be considered for a very short duration.

The mortality rate from staphylococcal scalded skin syndrome (SSSS) in children is very low (1-5%), unless associated sepsis, in adult it is higher (as high as 50-60%).

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