Staphylococcal scalded skin syndrome (Ritter disease) most commonly affects infants and children less than 5 years of age and is caused by an exfoliative exotoxin-producing strain of S aureus. Initial presentation includes fever, malaise, and irritability following an upper respiratory infection with pharyngitis or conjunctivitis. Patients first develop a diffuse faint blanching erythematous rash that is tender to the touch. Crusting around the mouth, eyes, and neck may be seen. Within 2 to 3 days, flaccid blisters and bullae develop, especially in flexor creases and the buttocks, hands, and feet. These bullae are sterile. In some patients, widespread desquamation occurs. The differential diagnosis includes toxic epidermal necrolysis, exfoliative erythroderma, bullous erythema multiforme, bullous pemphigoid, bullous impetigo, sunburn, enterovirus, drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, acute mercury poisoning, toxic shock syndrome, and epidermolysis bullosa.
Management and Disposition
If staphylococcal scalded skin syndrome is suspected, obtain cultures from the blood, urine, nasopharynx, abnormal area of skin, or other suspected foci of infection. Treatment is directed to eradicate S aureus, thus terminating the production of toxin. Semisynthetic penicillinase-resistant penicillins or clindamycin should be used intravenously. Consider the addition of vancomycin in areas with a high prevalence of MRSA. Admission is usually necessary, especially in young infants. This age group requires careful attention to fluid and electrolyte losses and the prevention of secondary infection of the denuded skin. Management of pain associated with the rash may require opioid analgesics. Children may develop a 2nd period of desquamation during the first 10 days of the illness.
The wrinkling or peeling of the upper layer of the epidermis (pressure applied with a Q-tip or gloved finger) that occurs within 2 or 3 days of the onset of this illness is known as Nikolsky sign.
The fluid in the bullae of staphylococcal scalded skin syndrome is sterile. The toxin is produced at a remote site and delivered to the skin via the bloodstream.
Infants with large body surface area involvement are at risk for hypothermia and fluid/electrolyte losses.
Corticosteroids are contraindicated in the treatment of staphylococcal scalded skin syndrome.
Staphylococcal Scalded Skin Syndrome. Toddler with diffuse macular peeling eruption consistent with scalded skin syndrome from S aureus. (Photo contributor: Judith C. Bausher, MD.)