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December 1, 2008

Picture of the Month—Diagnosis

Arch Pediatr Adolesc Med. 2008;162(12):1190. doi:10.1001/archpedi.162.12.1190

Denouement and Discussion: Staphylococcal Scalded Skin Syndrome

A clinical diagnosis of staphylococcal scalded skin syndrome (SSSS) was made based on the diffuse, tender erythroderma and increased erythema of the skin creases (Figure 1). The features distinguishing this from streptococcal scarlet fever were the skin being tender to the touch and the absence of strawberry tongue or palatal exanthem. The patient began receiving intravenous clindamycin phosphate on admission for a clinical diagnosis of SSSS. During the next couple of days the pain decreased, exfoliation of the skin was seen around the eyes and mouth, and the entire skin gradually underwent desquamation, further confirming our diagnosis (Figure 2). Her recovery was uneventful, without secondary skin infection.

Figure 1. 
Five-year-old girl with diffuse erythema of the face, neck, chest, and right upper extremity. Desquamation of the skin on the ear, the corner of the mouth, and the chest is also seen.

Five-year-old girl with diffuse erythema of the face, neck, chest, and right upper extremity. Desquamation of the skin on the ear, the corner of the mouth, and the chest is also seen.

Figure 2. 
Another patient, with desquamation of the skin around the eyes, lips, and neck.

Another patient, with desquamation of the skin around the eyes, lips, and neck.

Staphylococcal scalded skin syndrome is a rare dermatologic disorder associated with staphylococcal infection. Mockenhaupt et al1reported an incidence of 0.09 to 0.13 cases per 1 million people. After the initial prodrome of conjunctivitis or sore throat, a tender rash that is erythematous, diffuse, and usually most apparent in the flexural areas appears. The symptoms resolve in 2 weeks and the affected areas heal over without scarring.2

Stevens-Johnson syndrome, toxic epidermal necrolysis (TEN), cellulitis, erysipelas, and scarlet fever should be considered in the differential diagnosis. The most important dermatologic condition to be considered is TEN, as it has an overall mortality rate of 30%,3whereas SSSS has a mortality rate of about 4% in children.2Toxic epidermal necrolysis is usually due to a drug reaction commonly caused by aromatic anticonvulsants, lamotrigine, sulfonamide antibiotics, allopurinol, dapsone, or piroxicam.4Staphylococcal scalded skin syndrome usually occurs in children, and 98% of cases are in children aged 6 years or younger.2The distinguishing clinical features between TEN and SSSS are the presence of mucosal involvement and the presence of the Nikolsky sign (blisters spread easily on application of horizontal, tangential pressure to the skin) in only affected areas in TEN, whereas in SSSS there is no mucosal involvement and the Nikolsky sign is also present in areas that are not visibly affected.5

While infection with Staphylococcus aureusis the underlying cause of SSSS, the exotoxins ETA and ETB are responsible for the clinical symptoms in SSSS. The ETA binds to fillagrin in the granular layer. This binding disrupts the desmosomes in the epidermal layer, which then results in splitting of the epidermis.2Young children do not have antibodies to these toxins. In a study by Patel and Finlay,2only 41% of children aged 2 to 5 years had antibodies to ETA, whereas 91% of adults older than 40 years had antibodies to ETA. A skin biopsy is the gold standard for diagnosis. Histopathologically, TEN has epidermal necrosis with dermal sparing, whereas SSSS will show intraepidermal and subcorneal division.6

The most important consideration in the management of SSSS is in eradicating the underlying infection and concurrent supportive care. Clindamycin can help eliminate staphylococcal disease in infection with susceptible strains, and there is also evidence that it may inhibit toxin formation.7,8In addition to eradicating the focus of infection, antibiotics can help in preventing superinfection, which may occur secondary to the breakdown of the epidermal barrier. The prevention of superinfection is of high priority because the risk of mortality increases greatly. The antibiotics should be administered intravenously when symptoms are diffuse and widespread.6Early recognition and prompt supportive therapy along with antimicrobial therapy improve outcomes in patients with SSSS.

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Correspondence:Deepa Mukundan, MD, Division of Infectious Diseases, University of Toledo College of Medicine, 2222 Cherry St, Ste 2300, Toledo, OH 43608 (deepa.mukundan@utoledo.edu).

Accepted for Publication:August 1, 2008.

Author Contributions:Study concept and design: Chang and Mukundan. Acquisition of data: Chang and Mukundan. Drafting of the manuscript: Chang and Mukundan. Critical revision of the manuscript for important intellectual content: Mukundan. Study supervision: Mukundan.

Financial Disclosure:None reported.

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