Staphylococcal Scalded Skin Syndrome

Following an uncomplicated pregnancy, a 30-year-old gravida 2, para 1 mother delivered a term infant boy. The neonate's Apgar scores were 7 at 1 minute and 9 at 5 minutes. Birth weight was 3.2 kg (7.1 lb); length, 50 cm (19.7 in).

A yellow, foul-smelling discharge was noted in the infant's umbilicus when he was 10 days old. During the next several days, diffuse erythema of the skin and flaccid bullae developed; the bullae ruptured after 1 day, and peeling of the skin followed. Nikolsky sign was positive.

Drs Alexander K. C. Leung and Justine H. S. Fong of Calgary, Alberta, diagnosed staphylococcal scalded skin syndrome (SSSS), or Ritter disease. SSSS primarily affects infants; it is rarely seen in older children and adults. The predominant causative agent is phage group 2 staphylococci, particularly strains 55 and 71 that elaborate epidermolytic or exfoliative toxins A or B. Foci of infection include the nasopharynx, umbilicus, skin, conjunctivae, and blood.

Within a few days of the initial infection, diffuse erythema of the skin and marked skin tenderness develop. The erythematous skin may rapidly acquire a wrinkled appearance and, in some patients, flaccid blisters and erosions arise.

At this stage, areas of the epidermis can separate in response to gentle shear force (Nikolsky sign). The desquamative phase begins after 2 to 5 days of cutaneous erythema. Healing occurs without scarring in 10 to 14 days.

Since the staphylococci are usually penicillin resistant, administer penicillinase-resistant penicillin-such as nafcillin or oxacillin-parenterally; oral agents may be given for localized infection.

First- and second-generation cephalosporins (eg, cephalothin or cefuroxime) and clindamycin also are effective.