Épidermolyse toxinique staphylococcique du nouveau-né

Staphylococcal scalded skin syndrome (SSSS) is a dermatological condition caused by Staphylococcus aureus. The disease presents with the widespread formation of fluid-filled blisters that are thin walled and easily ruptured, and the patient can be positive for Nikolsky's sign. Ritter's disease of the newborn is the most severe form of SSSS, with similar signs and symptoms. SSSS often includes a widespread painful erythroderma, often involving the face, diaper, and other intertriginous areas. Extensive areas of desquamation might be present. Perioral crusting and fissuring are seen early in the course. Unlike toxic epidermal necrolysis, SSSS spares the mucous membranes. Children with SSSS may exhibit fussiness or irritability, tiredness, fever, redness of the skin, easily broken fluid-filled blisters that leave an area of moist, tender, painful skin, and large sheets of the top layer of skin that easily peel away. The condition is most common in children under 6 years, but can be seen in adults who are immunosuppressed or have kidney failure. The syndrome is induced by epidermolytic exotoxins (exfoliatin) A and B, which are released by S. aureus and cause detachment within the epidermal layer, by breaking down the desmosomes. One of the exotoxins is encoded on the bacterial chromosome, while the other is encoded on a plasmid. These exotoxins are proteases that cleave desmoglein-1, which normally holds the granulosum and spinosum layers together, similar to the pathophysiology of the autoimmune skin disease, pemphigus vulgaris. SSSS is a clinical diagnosis. This is sometimes confirmed by isolation of S. aureus from blood, mucous membranes, or skin biopsy; however, these are often negative. Skin biopsy may show separation of the superficial layer of the epidermis (intraepidermal separation), differentiating SSSS from TEN, wherein the separation occurs at the dermo-epidermal junction (subepidermal separation). SSSS may be difficult to distinguish from toxic epidermal necrolysis and pustular psoriasis.