SSSS is an acute, disorder caused by Staphylococcus aureus that manifests as a faint erythematous eruption which begins around the mouth and in body folds. The skin rapidly becomes tender with crusting around the mouth, eyes and neck. Flaccid blisters appear and rupture, leaving a moist, red or crusted erosive surface. Gentle pressure to the skin of an affected infant can shear off the epidermis (Nikolsky's sign) which folds like tissue paper. SSSS is uncommon after 5 years of age.

Differential diagnosis

Toxic epidermal necrolysis (TEN) is often confused with SSSS, however SSSS is more superficial with fine scaling. The two can be differentiated histologically as SSSS shows no dermal necrosis or inflammatory cells unlike TEN. SSSS may also resemble regular burns, sunburn, toxic shock syndrome or streptococcal scarlet fever.

Pathophysiology

S. aureus of phage group II elaborates a toxin that leads to injury of keratinocytes and results in intraepidermal separation of the cells within the granular layer and subsequent shedding of the entire granular layer and stratum corneum when minor trauma occurs. The main reason why the neonatal population is affected the most often is because their immature kidneys do not excrete the toxin and the toxin is transported hematogenously to the skin. In addition, neonates do not have specific antibody to the toxin.

Treatment

The patient can be treated as an outpatient with anti-staphylococcal antibiotics. A child who is more ill may need to be hospitalized. It is important to emphasize to parents that the lesions of SSSS are unlike burns as they do not scar.