Epidermal detachment after diffuse erythema in the infant

Specialty: Pediatrics.

• Staphylococcal scalded skin syndrome
• Ritter's disease
• positive Nikolsky sign

Why it occurs

• Staphylococcal scalded skin syndrome or SPEE (generalized exfoliative skin reaction mediated by epidermolytic or exfoliative toxins A and B secreted by specific strains of Staphylococcus aureus, which bind and selectively cleave desmoglein 1, a cell adhesion molecule located in the desmosomes of the granular layer of the epidermis)
• Toxic Epidermal Necrolysis or TEN / Stevens-Johnson Syndrome (severe delayed hypersensitivity reaction induced by drugs such as sulfonamides or anticonvulsants, characterized by keratinocytic necrosis of the entire thickness of the epidermis with involvement of multiple mucous membranes)
• Hereditary epidermolysis bullosa (genetic disorder of skin fragility that causes blisters and skin detachment due to minimal trauma from birth)
• Extensive thermal burn, accidental or due to physical abuse.

Initial workup

Meticulous clinical diagnosis is of utmost urgency. Sampling for smears, Gram stain, and bacteriological culture of possible primary foci of staphylococcal infection (such as conjunctival exudate, nasopharyngeal swab, smear from the umbilicus, external auditory canal, or any exudative skin wound; SPEE blisters are typically sterile as the injury is mediated by hematogenous toxin). Emergency paired blood cultures. Frozen skin biopsy (useful to quickly differentiate SPEE -with superficial intraepidermal subcorneal cleft- from NET -with full-thickness epidermal necrosis and dermal infiltrate-).

Standard management

• The treatment of moderate-severe SPEE should be in-hospital and intravenous, similar to the management of a severely burned patient. — protective isolation, meticulous fluid and electrolyte balance, and skin support care without adhesive bandages). Cloxacillin or Nafcillin (first-line penicillinase-resistant beta-lactam antibiotics; cloxacillin dose of 100 to 150 mg/kg/day intravenously divided every 6 hours
• Cefazolin — first generation cephalosporin as an effective alternative; doses of 50 to 100 mg/kg/day IV
• Clindamycin — added synergistically for its ability to inhibit the synthesis of staphylococcal toxins at the bacterial ribosomal level; dose of 20 to 40 mg/kg/day IV divided every 6-8 hours
• Vancomycin (indicated in case of suspected or confirmed methicillin-resistant Staphylococcus aureus; doses of 40 to 60 mg/kg/day IV). Avoid the use of corticosteroids in SPEE as they depress immunity and worsen the prognosis.