2.2.6 Staphylococcal scalded skin syndrome (SSSS)

Ritter von Rittershain disease (1878), Staphylogenic Lyell Syndrome (1956); Ritter`s disease; staphylogenic pemphigus neonatorum; Dermatitis exfoliativa neonatorum.

SSSS: affects up to 56 children out of 100,000, elderly are rarely involved.

SSSS: the acute exfoliative dermatitis is primarily seen in small children following staphylogenic infections with lysophage group II exfoliative toxins ETA A and B. STSS: the acute S. aureus toxin.

Staphylococcal Scalded Skin Syndrome (SSSS) is a toxin-mediated epidermolytic dermatosis and characterized by erythema and widespread loss of the superficial epidermal layers, resembling a burn. Mostly Staphyloccocus aureus isolates of the lyso (phage) group II are responsible. Products are ETA-A, -B and –D, which show hematogenous spread. The split in the epidermis is beneath the stratum corneum within the stratum granulosum followed by an acantholysis similar to pemphigus. The split takes place at this precise point because the exfoliative toxins act as serin proteases and damage desmoglein 1.

Following a staphylococcal infection in SSSS, initially a widespread erythema resembling scarlet fever appears followed within 24 to 48 hours by small unstable blisters which quickly become eroded and lead to widespread skin loss similar to grade II burns. In the acute course, the children are systemically ill. Nikolski sign is positive. Re-epithelisation takes place a week after desquamation. No scarring.

There are two clinical subtypes in SSSS,

• one in the first three months of life is called staphylogenic pemphigoid of the newborn, and,

• the other in older infants appearing as a staphylogenic Lyell-syndrome. Often an otitis, a pharyngitis or a conjunctivitis are preceding the skin manifestations. In the elderly often renal insufficiency or immune deficiency predispose to the disease.

Intensive care laboratory data regarding kidney, liver and blood, minerals, albumin and thorough investigations of bacterial strains are necessary.

Subcorneal blisters and bullae and acantholysis are the hallmarks of SSSS. An acantholytic cleft in the upper Stratum granulosum and a blister roof which only contains stratum corneum can be seen. (This is in contrast to TEN, in which one sees full-thickness epidermal damage).

Rapid onset within hours to two days course. In the elderly the mortality is high (up to 40%).

Sepsis, pneumonia, acute pulmonary distress syndrome. The high mortality in the elderly up to 40%, in children about 3-5 %.

History, clinical feature, histology (frozen section of blister roof), microbiology.

• TSST

• TEN (medication-induced)

• epidermolysis bullosa

• scarlatina

• chronic bullous disease of childhood (linear IgA disease)

• Kawasaki syndrome

Children and adults should be admitted immediately to an intensive care unit. Antibiotics covering penicillinase resistant staphylococci according to resistogram are mostly cefotaxime, flucloxacillin and clindamycin; immunoglobulins are recommended. Fluid replacement as in burn patients, elimination of staphylococcal foci; other antimicrobial disinfectant therapy (baths, compresses). Paracetamol, no NSAIDs; the most important means of controlling STSS / GAS disease and its sequelae is prompt identification and treatment of infections. Intravenous antibiotics like clindamycin or azithromycin may be first choice of systemic antibiotic treatment.