Synonym: Ritter syndrome, dermatitis exfoliativa neonatorum
Toxin: exfoliatoxin, which results in bulla formation: within the granular cell layer of the epidermis
Clinical features:
(1) Most cases occur in infants and children < 5 years old, although it can occur in adults.
(2) The patient develops a localized infection with Staphylococcus aureus (urinary tract, umbilicus in a neonate, nasopharynx, other). Toxin is absorbed into the bloodstream.
(3) The patient may present with fever, irritability and marked cutaneous tenderness.
(4) The patient develops a scarlatiniform eruption, with accentuation in flexural creases and around the mouth.
(5) Multiple large bullae are develop over the skin surface (generalized).
(6) Nikolsky's sign (easy separation of the outer portion of the epidermis while sliding a finger over the skin surface) is positive in skin adjacent to the bullae..
(7) Large amount of fluid and electrolytes can be lost through denuded areas of skin.
(8) A small percentage of patients may develop a sepsis syndrome with organ failures.
(9) Desquamation of the skin follows and may be accompanied by loss of hair and nails.
Laboratory features:
(1) Blood cultures are usually negative in children but may be positive in adults.
(2) Cultures of intact bullae are characteristically negative.
A variant syndrome (scarlatiniform) may have all of the clinical features except the formation of bullae.
Differential diagnosis:
(1) toxic epidermal necrolysis (TEN)
(2) toxic shock syndrome
(3) Kawasaki syndrome (mucocutaneous lymph node syndrome)
(4) other bullous diseases
