[Gram-positive septic-toxic shock with bullae. Intraepidermal splitting as an indication of toxin effect]

A fourteen-year-old girl with acute otitis media died from gram positive sepsis and toxic shock despite intensive treatment. The definitive bacteriological results showed positive cultures for both S. aureus and S. pyogenes serotype A. In vitro the bacteria produced the bacterial superantigens TSST-1, enterotoxin A, enterotoxin C (S. aureus) and erythrogenic toxin C (S. pyogenes). The patient presented with large flaccid sterile bullae on her chest and arms as well as necrotizing fasciitis. Tzanck test showed keratinozytes without necrosis and no inflammatory cells. Frozen-section and conventional skin biopsy specimens revealed subcorneal intraepidermal cleavage. These cytological and histological findings are those of staphylococcal scalded skin syndrome (SSSS) and differ from bullous erysipelas or toxic epidermal necrolysis (TEN). Therefore bacterial exotoxins are most likely responsible for the intraepidermal blistering in our case just as in SSSS. Bullae are an unfavorable prognostic sign in gram positive toxic shock syndrome. Both Tzanck test and frozen-section biopsy are easy to perform and useful in the early and rapid recognition of gram positive bullous toxic shock syndrome.