Staphylococcal scalded skin syndrome (SSSS) is a potentially lethal complication of a Staphylococcus aureus infection. Caused by a toxin emitted by the bacterium, rather than actual bacterial components, this syndrome leads to broad regions of exfoliating skin and is mainly observed in immunocompromised patients, newborns, infants and young children. SSSS is also known as Ritter's disease.
The first lesions are formed superficially and with crust. In the following 24 hour period, erythema and localized pain begin to appear, alongside a sensitivity of the skin in the affected region and a transparent appearance. This clinical manifestation develops in distant regions as well and within the next 24 hours, fluid-filled blisters (bullae) emerge, which easily cleave, causing a scalded appearance and a sheet-like exfoliation of the epidermis. The patients are many times positive for the Nikolsky sign, which involves the application of gentle pressure in non-bullous locations and the subsequent rapid formation of a blister, as the superficial epidermis can be easily dislodged.
Bullae tend to form in intertriginous areas, such as the groin, armpits, buttocks, etc. and systemic complications (fever, fatigue, chills, lethargy) arise within 36-72 hours after the onset of the original infection . Electrolyte imbalance, dehydration and sepsis are direct results of the impairment of the skin fortification caused by SSSS.
SSSS is diagnosed mainly clinically , but in order for a definitive diagnosis to be established, a biopsy needs to be harvested. A thorough histological examination will reveal a non-inflammatory superficial breach of the epidermis, which distinguishes SSSS from another complication of a staphylococcal infection, toxic epidermal necrolysis. Cultures can also be harvested from areas of possible primary infection, but they are not of much use, as they are often negative. No culture should be harvested from the bullae themselves, because they are sterile.
Upon the diagnosis of SSSS or increased clinical suspicion, beta-lactamase resistant anti-staphylococcal antimicrobials must be promptly administered IV .
Therapeutic schemes and dosages include:
The schemes above are given until some improvement is observed. Once this has been achieved, administration of the following is required:
Vancomycin should be chosen when there is a high suspicion of infection with Methicillin-resistant S. aureus (MRSA). Corticosteroids are strictly contraindicated . Demulcent lotions can also be applied to prevent further dehydration from the ruptured epidermis.
The mortality rate of SSSS in children is 4% and mainly depends on the extent of the affected skin . It is easily treated, with the bullae responding well to antimicrobial medications and absence of scarring . The syndrome poses risks for newborn patients, as their treatment is certainly more challenging. In cases of adult SSSS, prognosis is not as favorable, with mortality rates being as high as 60%.
S. aureus is a part of the skin's natural flora, colonizing the nasal cavity, the conjunctival regions and many other regions . While it does not always cause infections, it is potentially pathogenic, leading to:
S. aureus is classified as a commensal organism and in order for the body to maintain such a relationship with a potential pathogen, the immune system must be mature. The pathogen's toxins are also excreted via the kidneys. This is why SSSS is common mainly amongst children, whose immune system has not fully developed yet, and adults with immunodeficiency, immunosuppression or renal failure.
Staphylococci can be transmitted through simple skin contact, saliva or objects touched by a person colonized with it, naturally even in the absence of an active infection.
The greater majority of patients (98%) affected by SSSS involves newborns and children younger than 6 years old. Mortality rates are relatively low, estimated around 1-5% in young patients and death usually occurs due to another coexisting condition or septic phenomena. It is rarely observed in adults, but patients that do develop the syndrome run a high risk of succumbing to it, with adult mortality rates being astoundingly high, approximately 60%. In adults, increased mortality rates can be explained by a higher probability of underlying pathologies, sepsis, electrolyte imbalance and severe dehydration caused by the scalded skin. Incidence has been calculated between 0-09-0.13/1.000.000 individuals .
In general, the adult population has developed a greater amount of antibodies against the exfoliating exotoxin responsible for the staphylococcal scalded skin syndrome, in contradistinction to younger individuals whose immune system is not fully matured. Therefore, newborns or young children are more prone to an SSSS. Furthermore, the kidneys also mediate the toxin's excretion from the organism; should a patient suffer from renal failure, higher concentrations of the toxins are accumulated, making a staphylococcal infection and its complications a lot more probable.
Children of both sexes are affected equally, whereas adult males tend to be affected twice as much as adult women.
The staphylococcus aureus bacterium is an organism found in many locations of the human body, including the skin, nasal cavities and eyes. It can be a pathogenic organism with various mechanisms, which, among others, involve the release of specific toxins.
The type A and B toxins released from 5% of the various staphylococcal strains are responsible for the staphylococcal scalded skin syndrome. They assume a protease-like function, impairing the adhesion capacity of desmosomes, by attacking desmoglein-1 ; the latter is one of the adhesive proteins involved in the attachment of one epidermal cell to another. In this way, epithelial cellular bonds are destroyed and bullae are allowed to form, alongside skin epidermolysis.
Common locations from which the original infection arises include the umbilicus, nose or oral cavity . The ETA and ETB toxins are released from the microorganism and are transferred to distant locations via the bloodstream, or act locally; thus, they cause a breach of the epidermis beneath the granular cell layer. It is believed that the two possibilities of toxin transfer characterize two different types of SSSS: the regional (localized) form, where the bullae center around the original site of infection and a widespread form, where the bullae form at distant locations, when compared to their site of origination, due to hematogenous transfer.
S. aureus subtypes 3A, 3B, 3C, 55 and 71 belong to the phage lytic IIS group and were believed to be the ones exclusively responsible for the production of the exfoliating exotoxins. During the last years, it has been clarified that all phage groups can produce the ETA and ETB toxins.
Following basic hygienic measures helps to eliminate primary staphylococcal infections and is the first step towards preventing the onset of a dangerous complication such as SSSS. Secondly, any diagnosed staphylococcal infections should be treated prudently and promptly, in order to limit the extent of the infection. Lastly, in cases of immunosuppressed patients or other patients running an increased risk of developing SSSS, carriers of the bacterium should be diagnosed and treated prophylactically.
The staphylococcal scalded skin syndrome (SSSS) is caused by the bacterium S. aureus. It is a result of infection with certain bacterial strains that are capable of producing the exfoliating exotoxin and arises as a complication of the primary infection. Clinical manifestations include soft bullae commonly located on intertriginous areas of the body, which are followed by erythema and are easily cleaved, causing skin exfoliation and a scalded appearance. Usually, a 48-hour period suffices for the bullae to develop to the full extent and broad regions of the body are affected. The patients can also be positive for Nikolsky sign.
The exfoliating staphylococcal exotoxin, which is responsible for SSSS, adversely affects the desmosomal function: it destroys the desmoglein-1 complex, namely the complex responsible for the adhesion of epithelial cells, thus creating a breach which leads to the formation of bullae. SSSS is a severer, more generalized form of bullous impetigo, also caused by a staphylococcal infection and it is believed that the development of SSSS or bullous impetigo is directly dependent on the concentration of the released toxin and its potential systematic spread.
Staphylococcal scalded skin syndrome is a complication primarily affecting newborn, infants and children rather than adults, with the reported cases amongst the adult population being estimated at circa 50. Although in adults it is associated with immunosuppression and renal failure, there are subgroups of patients who are completely disease-free, other than the staphylococcal infection. Mortality rates are relatively low for young individuals (circa 4%), but extremely high for adults (60%) .
Staphylococcal scalded skin syndrome is a potentially lethal complication of a bacterial infection with specific strains of staphylococcus. It is most commonly observed in newborn children, infants and young children, as well as people with kidney failure or a weak immune system due to an underlying condition or treatment with certain drugs. SSSS causes blisters in the skin, which rupture, leading to exfoliation and a scalded appearance.
SSSS is caused by strains of the S. aureus bacterium which produce a specific toxin called exfoliating toxin. These toxins attack the proteins that are responsible for the bonds between the skin cells; in this way, cells are no longer connected to each other, fluid finds its way through the surface from the lower layers of the skin and blisters form. These blisters rupture easily and the skin peels off in sheets. The very same toxin that causes SSSS, also causes a similar condition called bullous impetigo, believed by many to be a different aspect of the SSS syndrome.
What are the common symptoms of SSSS?
Given that SSSS is in fact an infection, it starts with a fever and reddish skin. Blisters start to form after a day. Other possible symptoms include the formation of crust on the infection site, pain, weakness and a positive Nikolsky sign. The Nikolsky sign is a simple test, where gentle pressure and rubbing are exerted in an area of the skin (usually one that lies over a bone) and this procedure results in the prompt formation of a blister.
After the skin has started to exfoliate, the symptoms that may still persist are usually fever, chills and weakness.
Newborns often exhibit primary infections in the diaper area or around the umbilical cord. Older children often exhibit such lesions on their arms, legs, and trunk.
How is SSSS diagnosed?
A physician will diagnose SSSS based on the clinical manifestations. A detailed medical history, clinical examination, blood tests and possibly a biopsy taken from the site of the suspected infection will help your doctor establish a definite diagnosis of staphylococcal scalded skin syndrome.
Most patients recover quickly from SSSS, children in particular. Treatment details always depend on the age and general health status of the patient. Usually all patients require hospitalization in order to treat the complication and are first treated with antibiotics administered intravenously, followed by medication taken orally. Patients also receive supportive treatment, such as fluids, painkillers if needed, and soothing lotions to minimize dehydration through the exfoliated skin. Physiotherapy is also an option from which several patients can benefit a lot, because SSSS commonly affects the flexures, leading to a self-imposed immobility when patients feel uncomfortable walking.