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Staphylococcal scalded skin syndrome (SSSS) is an acute epidermolysis caused by a staphylococcal toxin. Infants and children are most susceptible. Symptoms are widespread bullae with epidermal sloughing. Diagnosis is by examination and sometimes biopsy. Treatment is antistaphylococcal antibiotics and local care. Prognosis is excellent with timely treatment.
SSSS almost always affects children < 6 yr (especially infants); it rarely occurs in older patients unless they have renal failure or are immunocompromised. Epidemics may occur in nurseries, presumably transmitted by the hands of personnel who are in contact with an infected infant or who are nasal carriers of Staphylococcus aureus. Sporadic cases also occur.
SSSS is caused by group II coagulase-positive staphylococci, usually phage type 71, which elaborate exfoliatin (also called epidermolysin), a toxin that splits the upper part of the epidermis just beneath the granular cell layer (see also Gram-Positive Cocci: Staphylococcal Infections). The primary infection often begins during the first few days of life in the umbilical stump or diaper area; in older children, the face is the typical site. Toxin produced in these areas enters the circulation and affects the entire skin.
Symptoms and Signs
The initial lesion is usually superficial and crusted. Within 24 h, the surrounding skin becomes painful and scarlet, changes that quickly spread to other areas. The skin may be exquisitely tender and have a wrinkled tissue paper–like consistency. Large, flaccid blisters arise on the erythematous skin and quickly break to produce erosions. Intact blisters extend laterally with gentle pressure (Nikolsky's sign). The epidermis may peel easily, often in large sheets. Widespread desquamation occurs within 36 to 72 h, and patients become very ill with systemic manifestations (eg, malaise, chills, fever). Desquamated areas appear scalded. Loss of the protective skin barrier can lead to sepsis and to fluid and electrolyte imbalance.
Diagnosis
Diagnosis is suspected clinically, but confirmation usually requires biopsy (frozen section may give earlier results). Specimens show noninflammatory superficial splitting of the epidermis. In children, skin cultures are seldom positive; in adults, they are frequently positive. Cultures should be taken from the nose, conjunctiva, throat, and nasopharynx.
Differential diagnosis
Differential diagnosis includes drug hypersensitivity, viral exanthemas, scarlet fever, thermal burns, genetic bullous diseases (eg, some types of epidermolysis bullosa), acquired bullous diseases (eg, pemphigus vulgaris, bullous pemphigoid), and toxic epidermal necrolysis (see Table 2: Bacterial Skin Infections: Differentiating Staphylococcal Scalded Skin Syndrome (SSSS) and Toxic Epidermal Necrolysis (TEN) ; see Hypersensitivity and Inflammatory Disorders: Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)). Stevens-Johnson syndrome is characterized by mucosal involvement, which is absent in SSSS.
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Table 2
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| Differentiating Staphylococcal Scalded Skin Syndrome (SSSS) and Toxic Epidermal Necrolysis (TEN) |
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Feature
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SSSS
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TEN
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Patients affected
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Infants, young children, immunocompromised adults
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Older patients
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Patient history
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Recent staphylococcal infection
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Drug use, renal failure
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Level of epidermal cleavage (blister formation)*
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Within the granular cell (outermost) layer of the epidermis
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Between the epidermis and dermis or at the level of the basal cell
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*Determined by Tzanck test or by a frozen section of a fresh specimen.
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Treatment
With prompt diagnosis and therapy, death rarely occurs; the stratum corneum is quickly replaced, and healing usually occurs within 5 to 7 days after start of treatment.
Penicillinase-resistant antistaphylococcal antibiotics given IV must be started immediately. Nafcillin 12.5 to 25 mg/kg IV q 6 h for neonates > 2 kg and 25 to 50 mg/kg for older children is given until improvement is noted, followed by oral cloxacillin 12.5 mg/kg q 6 h (for infants and children weighing ≤ 20 kg) and 250 to 500 mg q 6 h (for older children). Corticosteroids are contraindicated. Topical therapy and patient handling must be minimized.
If disease is widespread and lesions are weeping, the skin should be treated as for burns (see Burns: Treatment). Hydrolyzed polymer gel dressings may be very useful, and the number of dressing changes should be minimized.
Steps to detect carriers and prevent or treat nursery epidemics are discussed elsewhere (see Infections in Neonates: Prevention).
Last full review/revision October 2007 by A. Damian Dhar, MD, JD
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