Staphylococcal scalded skin syndrome (SSSS)

Spectrum of superficial blistering skin disorder caused by the exfoliative toxins of Staphylococcus aureus.

History:

The clinical features of SSSS were first described over a century ago by a German physician, Baron Gotfried Ritter von Rittershain, who observed 297 such cases among young children in a 10-year period while working in a Czechoslovakian foundling asylum; he called it dermatitis exfoliativa neonatorum but was unable to determine its cause. The term “Ritter’s disease,” named after its describer, is still used when generalized SSSS occurs in neonates. “Pemphigus neonatorum” describes a milder, self-limiting disease of infants that produces a few blisters. S. aureus was first isolated in 1891 from a patient with pemphigus neonatorum by Almquist, who called the organism Micrococcus pemphigi neonatorum, and then by Winternitz in 1898.

The disease received little attention until 1956, when Lyell described a skin eruption that resembled scalded skin and called it toxic epidermal necrolysis (TEN). He speculated that the condition might be due to a circulating toxin that damages the skin and causes necrosis, although he was not aware that he was describing two distinct, unrelated conditions. In 1967, however, Lyell reviewed 128 cases that fitted his diagnostic criteria of TEN and found that almost one-quarter—all children under 10 years of age—presented predominantly with a staphylococcal infection. A link between staphylococcal infection in children and exfoliation was also observed by Lowney et al.

It soon became apparent that the exfoliation associated with S. aureus infection occurred specifically within the mid-epidermis whereas exfoliation that was not associated with staphylococcal infection occurred at the dermoepidermal junction. As a result, the former condition was termed SSSS and the latter was termed TEN. The etiology and pathogenesis of SSSS are very different from those of TEN, and although they may be clinically difficult to distinguish, correct diagnosis is essential because the treatment for the two conditions is different.

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Etiology

Causative organism:

• Coagulase-positive group II Staphylococcus aureus (especially strain 71)

Risk factors:

Due to their lack of immunity and inadequate renal clearance ability

• Children & neonates
• Adults with renal failure, immunologic deficiency, and other chronic illnesses

Epidermolytic toxins (ETs):

Epidermolytic toxins (ETs) released by Staphylococcus aureus, particularly ETA and ETB, are thought to lyse desmoglein-1, present on desmosomes located in the strata granulosum of the epidermis, causing a loss of cell-to-cell adhesion between the keratinocytes, finally leading to intraepidermal splitting

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Presentation

Clinical features of SSSS vary from localized blisters to severe exfoliation affecting over 90% of the entire body surface.

Prodromal features: .

SSSS usually follows localized staphylococcal infection of the skin, throat, nose, mouth, umbilicus, or gastrointestinal tract

• General malaise, fever, irritability
• Skin tenderness
• Other signs: Facial edema, conjunctivitis, and perioral crusting

Localized form: (mildest form)

Also known as bullous impetigo, tropical bullous impetigo, measles pemphigoid, and bullous varicella is characteristic fragile, thin-roofed, flaccid bullae are formed, which rupture easily to release fluid that varies from a thin, cloudy, amber liquid to purulent, opaque, white or yellow pus

• Mildest form in which the surrounding skin remains normal and there are no systemic symptoms or signs

Generalized form:

Widespread involvement of the entire skin surface usually following a localized infection

1. Prodrome: Fever, malaise, and lethargy, with poor feeding and irritability
2. Generalized, tender erythematous rash: Begins on head & neck and spreads to the rest of the body within a few days
3. Widespread exfoliation of skin: Within 24-48 h, blisters with fluid accumulation form all over body following which large area of skin surface peel off manifesting as exfoliation/desquamation
   • Nikolsky’s sign (+): Extension of blister and/or removal of epidermis on applying tangential pressure with finger on skin

Complications:

D/t poor temperature control and extensive loss of fluids from skin

• Hypothermia & dehydration
• Secondary skin infections
• Primary/secondary staphylococcal sepsis: Hypotension, tachycardia, neutropenia, and/or respiratory distress

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Diagnosis

Nikolsky’s sign:

Elicited by applying tangential pressure with a finger or thumb to the affected skin, peri-lesional skin, or normal skin in patients with suspected pemphigus

Skin swab culture:

Samples subjected to bacteriological confirmation and antibiotic sensitivities

Frozen skin biopsy:

CONFIRMATORY

Differential diagnosis:

• Stevens-Johnson Syndrome/toxic epidermal necrolysis (TEN): Pattern of skin peeling differs and TEN occurs due to drug reaction
• Staphylococcal scarlet fever: Fever that mainly children and the simple erythematous rash, without blisters or Nikolsky’s sign, which is observed on days 5-10 followed by desquamation.
• Pemphigus: Group of autoimmune disorders having blistering of the skin and/or mucosal surfaces

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Management

Supportive care:

• Rehydration
• Antipyretics (e.g., ibuprofen, aspirin, and paracetamol)
• Management of thermal burns
• Stabilization
• Parenteral antibiotics

Eradication of primary infection:

• Nafcillin, oxacillin, or vancomycin
• Clindamycin (inhibition of exotoxins )