Staphylococcal scalded skin syndrome (Ritter's disease…)

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.

• Rare condition
• Primarily affects infants and young children; (98% of patients are < 6 years of age)
• A few exceptional cases have been reported in adults with immunocompromise or chronic illness, however, SSSS remains very rare in adults.

References:^[1]

Epidemiological data refers to the US, unless otherwise specified.

• 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.
• :
• 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]

Initially

• Fever, malaise, and irritability
• Skin tenderness
• Diffuse or localized erythema, often beginning periorally
  • Localization
    • Infants: perianal, perineal, periumbilical
    • Children: extremities
    • Very ill children: generalized lesions

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]

• Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN)

Differential diagnoses of severe exfoliative skin conditions
	SSSS	SJS	TEN
Age of typical patient	Children < 6 years	Adults	Adults
Etiology	Infectious: S. aureus exfoliative toxins	Adverse drug reaction (Infectious etiologies exist but are less common)	Adverse drug reaction (Infectious etiologies exist but are less common)
Clinical features	Sloughing of skin, Nikolsky's sign Perioral erythema and crusting but no mucous membrane involvement	Sloughing of skin, Nikolsky's sign Mucous membrane involvement Typically < 10% of total body surface area	Sloughing of skin, Nikolsky's sign ≥ 2 mucous membranes involved > 30% of total body surface area
Biopsy	Intraepidermal blistering Lack of inflammatory infiltrate	Degeneration and blistering of stratum basale of epidermis → subepidermal blisters Eosinophils and mononuclear infiltrate in the papillary dermis	Eosinophilic full-thickness epidermal necrosis Cell-poor infiltrate, sparse, and with lymphocytes

References:^[3][8]

The differential diagnoses listed here are not exhaustive.

• Hospital admission
  • Necessary in most cases
  • Isolation recommended
  • Severe cases may be treated in the burn unit or intensive care unit.
  • Outpatient treatment is possible in older children who are eating and drinking well and have minimal skin involvement.
• 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
  • Fluid rehydration as indicated
  • Supportive skin care: emollients; , covering denuded areas
  • NSAIDs as indicated for pain and fever

Steroids are contraindicated, as the etiology of SSSS is infectious! (They are, however, indicated in SJS and TEN.)

References:^[1][5][7][9]