SSSS is a term which encompasses a blistering skin disease caused by the epidermolytic (or exfoliative) toxin (ET)-producing S. aureus. The pathogenesis of SSSS is due to the production of ETs, of which there are two different serotypes affecting humans, ETA and ETB. SSSS usually begins with localized infection of the conjunctivae, nares, perioral region, perineum, or umbilicus.
Removal of perioral crusts leaves behind radial fissures around the corners of mouth, resulting in the characteristic facial appearance of SSSS. Other infections that can serve as the initial nidus for SSSS include pneumonia, infective endocarditis, septic arthritis, or pyomyositis. Fever, malaise, irritability, and poor feeding subsequently progress, lead to generalized eruption.
The rash is characterized by erythema that enlarges to large, superficial fragile blisters that rupture easily and result in denuded, desquamating, erythematous, and tender skin. The eruption is most marked in flexural creases but can also sometimes involve the entire surface area of skin. The Nikolsky’s sign (progression of the blister upon gentle pressure on the edge of the bulla) is positive. The most reliable distinguishing feature of TEN is mucosal involvement that involves the corner of the mouth, conjunctivae, trachea, and genital mucosa, which is not seen in SSSS.
It is worth to be noted that the majority of blisters in SSSS are sterile, as they are caused by the hematogenous dissemination of the bacterial toxin and not by the spread of bacteria itself. Treatment of SSSS is directed at the eradication of toxin-producing staphylococci and thus terminating toxin production and its dissemination.
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