- Clinical science
Staphylococcal scalded skin syndrome (Ritter's disease…)
Summary
Staphylococcal scalded skin syndrome (SSSS) is a potentially serious acute skin condition caused by the exfoliative toxins of Staphylococcus aureus and typically affects infants and young children. It is usually preceded by a mucocutaneous staphylococcal infection, such as pharyngitis or bullous impetigo, though this preceding infection may go unnoticed by patients and other caregivers. Following systemic dissemination of toxins from the local infection, SSSS itself typically begins with skin tenderness, erythema, and fever. This is followed a day or two later by flaccid blisters and sloughing off of the superficial layer of skin to reveal moist, red tissue underneath, giving the area a “scalded”-looking appearance. Mucous membranes are spared. A presumptive diagnosis of SSSS is based on clinical findings. Biopsy is only performed in unclear cases and shows separation of the epidermis at the granular layer. Treatment involves the administration of antibiotics and potential intensive care monitoring. The prognosis is generally good, and blisters heal without significant scarring.
Epidemiology
- Rare condition
- Primarily affects infants and young children; (98% of patients are < 6 years of age)
References:[1]
Epidemiological data refers to the US, unless otherwise specified.
Etiology
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Pathogen: Staphylococcus aureus strains that produce exfoliative toxins
- Exfoliative toxins A and B have been shown to cleave desmoglein-1 in the granular layer of the epidermis, thereby disrupting keratinocyte attachments.
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Route of infection: dissemination of toxins from a local infection
- Following a staphylococcal infection elsewhere (e.g., skin, mouth, nose, throat, GI tract, or umbilicus). The initial infection may also be completely undetected.
- Following bullous impetigo
SSSS belongs to the spectrum of diseases mediated by specific staphylococcal toxins, which also includes bullous impetigo, toxic shock syndrome (TSS), and Staphylococcus aureus food poisoning. Unlike TSS, SSSS does not have systemic manifestations (e.g., liver, kidney, bone marrow, and CNS involvement)!
References:[2][3][4][1]
Clinical features
Initially
- Fever, malaise, and irritability
- Skin tenderness
- Diffuse or localized erythema, often beginning periorally
After 24–48 hours
- Flaccid, easily ruptured blisters that break to reveal moist, red skin beneath (i.e., with a “scalded” appearance) → widespread sloughing of epidermal skin
- Nikolsky's sign
- No mucosal involvement
- Cracking, and crusting is common
- Signs of shock (hypotension, tachycardia)
Stevens-Johnson syndrome and toxic epidermal necrolysis present with mucosal involvement, SSSS doesn't!
References:[1][5]
Diagnostics
The presumptive diagnosis of SSSS is made based on clinical findings. Cultures (e.g., blood or nasopharynx) are usually taken for confirming the diagnosis, and a biopsy may be performed to exclude suspected differential diagnoses, but is usually not required.
- History: localized staphylococcal infection; (e.g., pharyngitis, bullous impetigo)
- Laboratory tests: for confirming the diagnosis
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Biopsy: indicated in unclear cases, especially when TEN or SJS are suspected
- Intraepidermal fissure and blister formation at the granular layer
- Lack of inflammatory cell infiltrate
References:[1][6][7]
Differential diagnoses
- Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN)
Differential diagnoses of severe exfoliative skin conditions | |||
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SSSS | SJS | TEN | |
Age of typical patient |
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Etiology |
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Clinical features |
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Biopsy |
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References:[3][8]
The differential diagnoses listed here are not exhaustive.
Treatment
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IV antibiotics
- Penicillinase-resistant penicillins are the drug of choice: nafcillin, oxacillin
- In areas with high community-acquired MRSA; prevalence (or in patients who do not respond to treatment): vancomycin
- Supportive care
Steroids are contraindicated, as the etiology of SSSS is infectious! (They are, however, indicated in SJS and TEN.)
References:[1][5][7][9]
Complications
The complications faced by SSSS patients are similar to those of patients with burns, as both have a compromised skin barrier:
- Fluid and electrolyte imbalances
- Thermal dysregulation
- Secondary infections (e.g., pneumonia, sepsis)
References:[8]
We list the most important complications. The selection is not exhaustive.
Prognosis
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Mortality rate
- Children: < 5%
- Adults: > 60%
- Blisters heal without scarring, as skin cleavage is intraepidermal
References:[7][10]