2.2.7 Staphylogenic/Streptogenic Toxin Shock-Syndrom (STSS)

StaphTSS: Epidemiological studies conducted in the UK and Ireland reported an incidence rate for STSS of 0.38 per 100,000 children. Both children and adults cases of varying severity and death rates of up to 50-60% in septic shock forms, are described in the literature.

Around 50 cases per year of StreptTSS are reported in the US. The number of menstrual STSS cases in the USA is becoming lower, data for the non-menstrual subtype show a female: male ratio of 3:1.

Staphyloccocal TSS: the acute S.aureus toxin TSST-1 and several enterotoxins causes disease which gives high fever, myalgia, nausea, headache, vomiting, pulmonary distress and diffuse macular erythema followed by erythroderma and later exfoliation.

Streptococcal TSS is related to pyogenic group A streptococci.

In Staphylogenic Toxin Shock Syndrome (STSS) the causative agent is the Toxic Shock Syndrome-Toxin-1 and the enterotoxins SEA to SEO. STSS was confirmed according to 2011 CDC criteria, and blood cultures positive for Methicillin-sensitive Staphylococcus aureus (MSSA). In cases of streptococcal origin (Streptococcal Toxin Shock-Syndrome), the pyrogenic exotoxins (SPE) A, B and C are responsible. They act via unrestricted MHC-II binding as superantigens activating specific T-cells which lead then to TNF alpha,IL 1ß and IL-6 release. One differentiates two subtypes. STSS menstrual type related to toxin TSST-1 is linked to the use of tampons with accumulation of staphylococci during menstruation. The STSS non-menstrual type is related to toxins produced by specific S.aureus colonies appearing in infections such as bursitis, after intra-articular injections or infected arthropod stings / bites.

In STSS of staphylogenic or streptogenic origin the symptoms are systemic with high fever, vomiting and nausea, myalgia and myositis, headache, strawberry tongue and a widespread macular erythema extending to an erythroderma and followed by body skin desquamation, particularly at first on the palms and soles, also affecting mucous membranes in the mouth and vagina. Nail and hair loss may occur. Periportal hepatic inflammation, acute tubular necrosis, and the abnormal pulmonary findings of acute respiratory distress syndrome (ARDS) are life threatening symptoms. Cases of streptococcal TSS can be associated with necrotizing fasciitis.

There are two clinical subtypes in StaphTSS, one in the first three months of life is called staphylogenic pemphigoid of the newborn, and, the second in older infants as a staphylogenic Lyell-syndrome. Often an otitis, a pharyngitis or a conjunctivitis precede. In the elderly, renal insufficiency or immune deficiency often predispose to the disease.

Menstrual STSS is related to tampon use and staphylococcal toxin and non-menstrual STSS is related to staphylococcal and streptococcal infections of various origin.

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. (In contrast to TEN in which one sees full-thickness epidermal damage.) In STSS damage is seen in the whole epidermis and intraepidermal and subepidermal clefts, necrotic cells and apoptosis appear. Microthrombi of the capillaries occur. This is sometimes difficult to distinguish from TEN.

Rapid onset within hours to two days [7]. Both children and adults cases of varying severity and death rates of up to 50-60% in septic shock forms, are described in the literature.

Mortality 10-20% in the streptogenic type, menstrual STSS 2-5%, non-menstrual 8-11%.

Sepsis, pneumonia, acute pulmonary distress syndrome.

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

• 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 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. Whereas penicillin is very effective in mild Streptococcus pyogenes infection, it is less effective in severe infections because of its short postantibiotic effect, inoculum effect, and reduced activity against persisting-phase organisms. Emerging treatments for strep TSS include furthermore intravenous gamma-globulin.