A Rapidly Spreading, Painful, Erythematous Rash in a 3-Year-Old Girl
A previously healthy 3-year-old girl was admitted for assessment of a painful, rapidly progressing rash that had been present for 5 days. One day before the rash’s appearance, her mother had noted a “bug bite” on the girl’s right hand. Rhinorrhea and mild cough developed overnight.
The next afternoon, she complained of axillary pain when being lifted by her parents. Subsequently, a red rash was observed at the center of each underarm that was painful to the touch. Over the next 2 days, the rash spread quickly, first appearing in skin folds, then on her neck, back, groin, and face.
Before hospitalization, the patient had been seen by physicians on 3 occasions; oral corticosteroids and antihistamines were prescribed but resulted in no improvement. Pharyngeal and rectal swabs also had been collected, and test results for streptococcal antigen were negative.
On initial physical examination, the girl was afebrile with normal vital signs. Immediately evident was a widespread, maculopapular, blanching, erythematous rash. It was especially prominent in the intertriginous, cervical, and periorbital areas. An isolated circular area of rash was present on her left upper chest where an adhesive temperature probe had been attached the day before.
What is the cause of this girl’s cutaneous symptoms?
A. Stevens-Johnson syndrome
B. Staphylococcal scalded skin syndrome
C. Toxic epidermal necrolysis
D. Pemphigus foliaceus
(Answer and discussion on next page)
Answer: B, staphylococcal scalded skin syndrome
The Nikolsky sign was positive, and a small area of skin sloughing was visible on her nose. Perioral crusting and a linear red streak on her right temple also were noted. She had an intact oral mucosa. The rest of the physical examination findings were benign.
Results of a complete blood count showed mild leukocytosis and thrombocytosis. Antistreptolysin O titer was negative, and results of a basic metabolic panel were within normal limits.
This patient’s distinctive presentation on physical examination led to a presumptive diagnosis of staphylococcal scalded skin syndrome (SSSS), an acute dermatologic manifestation of infection with an exfoliative exotoxin produced by certain strains of Staphylococcus aureus.
Formerly known as Ritter’s disease, SSSS most often is seen in neonates and young children with S aureus infections, because their immature kidneys lead to decreased renal function and, consequently, decreased excretion of the exotoxin.1
Bacterial exfoliative toxin genes ETA, ETB, and ETD encode isoforms of a serine protease that has been experimentally demonstrated to cleave a component of epithelial tight junctions, desmoglein.2,3 This protein’s importance in maintaining the skin’s integrity is evidenced by its involvement in both pemphigus disorders and SSSS.4,5
The clinical course of SSSS is a painful, quickly spreading, erythematous and blanching rash that first appears at intertriginous sites and often is accompanied by upper respiratory symptoms and/or localized skin abscesses.6 A positive Nikolsky sign is uniformly present.7 Bullae grow and rupture, leaving the typical “scalded” appearance. Healing occurs without scarring owing to the intraepidermal nature of exfoliation.8
SSSS spares the oral mucosa, unlike toxic epidermal necrolysis or Stevens-Johnson syndrome, allowing differentiation among these conditions based on physical examination findings. Methicillin-sensitive and methicillin-resistant strains of S aureus have both been documented as carrying the exfoliative toxin genes.9,10
Fluid losses and bacterial superinfection are common complications affecting the management of patients with SSSS. Antibiotics targeting protein synthesis might decrease toxin production and thus are frequently employed. The prognosis generally is good, with no permanent scarring for most affected children.8
Our patient was treated with intravenous vancomycin and clindamycin. Intravenous fluids, topical mupirocin, and petroleum jelly also were utilized. A nasal swab sent for culture grew methicillin-sensitive S aureus after 48 hours.
Nicholas A. Klaiber, MD, is a first-year pediatric resident at Children’s Hospital of Richmond at VCU in Richmond, Virginia.
Hannah Thomason Fox is a third-year medical student at Virginia Commonwealth University School of Medicine in Richmond.
Clifton C. Lee, MD, is chief of pediatric hospital medicine and an associate professor in the Department of Pediatrics at Children’s Hospital of Richmond at VCU.
Kirk Barber, MD, FRCPC—Series Editor, is a consultant dermatologist at Alberta Children’s Hospital and clinical associate professor of medicine and community health sciences at the University of Calgary in Alberta.
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