(See also Overview of Bacterial Skin Infections.)
Staphylococcal scalded skin syndrome almost always affects children < 6 years (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.
Staphylococcal scalded skin syndrome 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 by targeting desmoglein-1 (see also 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.
The initial lesion is usually superficial and crusted. Within 24 hours, 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. Blisters are frequently present in areas of friction, such as intertriginous areas, buttocks, hands, and feet. Intact blisters extend laterally with gentle pressure (Nikolsky sign). The epidermis may peel easily, often in large sheets. Widespread desquamation occurs within 36 to 72 hours, 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 is suspected clinically, but confirmation usually requires biopsy (frozen section may give earlier results). Specimens show noninflammatory superficial splitting of the epidermis.
Cultures should be taken from the conjunctiva, nasopharynx, blood, urine, and areas of possible primary infection, such as the umbilicus in a neonate or suspect skin lesions. Cultures should not be taken from bullae because they are sterile, unlike in bullous impetigo, where cultures of the blister fluid will yield a pathogen.
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: Differentiating Staphylococcal Scalded Skin Syndrome (SSSS) and Toxic Epidermal Necrolysis (TEN) and see Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis (TEN)). The mucosal surfaces are spared in SSSS; however, they are affected in Stevens-Johnson syndrome and toxic epidermal necrolysis.
Differentiating Staphylococcal Scalded Skin Syndrome (SSSS) and Toxic Epidermal Necrolysis (TEN)
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 every 6 hours 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 every 6 hours (for infants and children weighing ≤ 20 kg) and 250 to 500 mg every 6 hours (for older children). Vancomycin, linezolid, or other drugs effective against MRSA should be considered in areas with a high prevalence of methicillin-resistant S. aureus (MRSA) or in patients failing to respond to initial therapy. Corticosteroids are contraindicated.
Emollients (eg, white petrolatum) are sometimes used to prevent further insensible water loss from ulcerated skin. However, topical therapy and patient handling must be minimized.
If disease is widespread and lesions are weeping, the skin should be treated as for burns. 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 prevention of neonatal hospital-acquired infection).
Generalized desquamation and systemic illness are most often toxic epidermal necrolysis in older patients and staphylococcal scalded skin syndrome (SSSS) in infants and young children (and occasionally in immunocompromised adults).
Do a biopsy and culture the conjunctiva, nasopharynx, blood, urine, and areas of possible primary infection, such as the umbilicus and suspect skin lesions.
Treat patients with antistaphylococcal antibiotics and, if disease is widespread, in a burn unit if possible.
Monitor and treat for complications similar to those that occur with burns (eg, fluid and electrolyte imbalance, sepsis).