Neonate twin with staphylococcal scalded skin syndrome from a renal source

OBJECTIVE: To understand the underlying mechanism of exfoliative toxins causing staphylococcal scalded skin syndrome or Ritter's Disease that predominantly affects newborns and infants, although it is sometimes found in adults. Staphylococcal scalded skin syndrome is typically diagnosed by the characteristic fluid-filled bullae together with superficial skin loss. A histopathological diagnosis may be made by looking for subcorneal acantholytic cleavage with minimal inflammation on biopsy, although this is not normally required. Exfoliative toxin A and B are both responsible for the "acantholytic" infection of Staphylococcus aureus as they target desmoglein-1 leading to loss of cell-to-cell cohesion and subsequent spread of infection. Other factors produced by S. aureus can cause a myriad of other problems including neutralization of antimicrobial peptides, inactivation of neutrophils, proteolysis, T-cell anergy, and immunosuppression. DESIGN: Individual care report. SETTING: Pediatric intensive care unit. PATIENT: We describe a normal male infant who was born at term and developed 100% total body surface area staphylococcal scalded skin syndrome on the 14 day of life with associated renal sepsis. INTERVENTIONS: After cultures from the lesions, bloodstream, and urine were obtained, intravenous Vancomycin and Ceftriaxone were commenced. The initial lesions increased in size over a 36-hr period to cover the entire body surface; this was associated with a decline in hemodynamic status. MEASUREMENTS AND MAIN RESULTS: Cultures from the urine and blood grew coagulase-positive S. aureus. An ultrasound scan revealed bilateral pyonephroses, which necessitated the placement of percutaneous nephrostomies with subsequent decompression of the collecting system. CONCLUSIONS: After the decompression hemodynamic status stabilized and over the ensuing 10 days, the patient made a full recovery with no scarring. No similar lesions were noticed on the infant's twin brother. We discuss the recent developments in understanding the underlying mechanism of exfoliative toxins causing staphylococcal scalded skin syndrome, review current treatment guidelines, and outline the need for new therapeutic options.