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Staphylococcal Scalded Skin Syndrome

SSSS

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.


Presentation

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 [10]. Electrolyte imbalance, dehydration and sepsis are direct results of the impairment of the skin fortification caused by SSSS.

Fever
  • SSSS should be considered in the differential diagnosis of immunocompromised adult patients with sudden onset of high fever and erythema.[ncbi.nlm.nih.gov]
Localized Pain
  • 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.[symptoma.com]
Epilepsy
  • The roles of epilepsy and antiepileptic medications are discussed as possible predisposing factors.[ncbi.nlm.nih.gov]
Erythema
  • There is a generalized tender erythema which commences on the head and neck, accompanied by fever, irritability, continuous cry and miserable look.[ncbi.nlm.nih.gov]
Nikolsky's Sign
  • Available from: Sir, Nikolsky's sign is an easily inducible and a valuable sign in clinical dermatology.[ijpd.in]
  • The erythema is followed by cleavage of the upper epidermis in a large sheets mainly in the head, neck and the flexures, with formation of bullae (Nikolsky sign). It is most common in infants and children under 5 years.[ncbi.nlm.nih.gov]
  • The patients can also be positive for Nikolsky sign.[symptoma.com]
Exfoliation of the Skin
  • We had three cases of SSSS with varied dermatological manifestations-diffuse/scarlitiniform erythema, generalized exfoliation, sand paper skin texture, flaccid bullae, erosions, seborrheic dermatitis like scaling and cracking in skin creases which can[ncbi.nlm.nih.gov]
  • There is marked epidermal exfoliation with the skin peeling off in sheets leaving exposed moist, bright-red, tender areas The Nikolsky sign (gentle stroking of the skin causes the skin to separate at the epidermis) is positive SSSS differs from the more[pcds.org.uk]
  • In young infants, and in those with renal impairment, these can accumulate and result in widespread exfoliation of the skin known as Staphylococcal scalded skin syndrome (SSSS). The initial infection is often minor or undetected.[starship.org.nz]
  • It is a syndrome of acute exfoliation of the skin typically following an erythematous cellulitis.[emedicine.com]
Flushing
  • Facial flushing with circumoral pallor is common. The rash fades within a week and is followed a week later by a superficial desquamation, especially in skin folds, on the face and in sheets from the hands and feet (may take 2 to 6 weeks).[healio.com]
Chronic Dermatitis
  • The ancillary nurse suffered from chronic dermatitis on her hands that favoured S. aureus carriage.[ncbi.nlm.nih.gov]
Facial Redness
  • Posterior Blepharitis • Inflammation of eyelids secondary to dysfunction of meibomian glands • Associations – Rosacea • Facial redness – Demodex mites • Affinity for hair follicles 18.[slideshare.net]
Cerebellar Ataxia
  • Staphylococcal scalded skin syndrome (SSSS), not previously recorded as a chronic disease, persisted for 2 years in a 50-year-old woman with epilepsy and cerebellar ataxia.[ncbi.nlm.nih.gov]
Ataxia
  • Staphylococcal scalded skin syndrome (SSSS), not previously recorded as a chronic disease, persisted for 2 years in a 50-year-old woman with epilepsy and cerebellar ataxia.[ncbi.nlm.nih.gov]
Kidney Failure
  • Adult SSSS is usually associated with immunosuppression, overwhelming sepsis, and kidney failure.[ncbi.nlm.nih.gov]
  • It rarely occurs in older people except for those with kidney failure or a weakened immune system. Like other staphylococcal infections, staphylococcal scalded skin syndrome is contagious.[merckmanuals.com]
  • Staphylococcal scalded skin syndrome is rare in adults, but can affect those who have kidney failure and immune deficiency, those on immune suppressant drugs or undergoing chemotherapy.[bad.org.uk]

Workup

SSSS is diagnosed mainly clinically [11], 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.

Anergy
  • Other factors produced by S. aureus can cause a myriad of other problems including neutralization of antimicrobial peptides, inactivation of neutrophils, proteolysis, T-cell anergy, and immunosuppression. Individual care report.[ncbi.nlm.nih.gov]
Blood Culture Positive
  • SSSS is usually associated with a trivial infective focus in the conjunctivae or the skin; however, severe infections, such as sepsis, do contribute to a low but appreciable fatality rate ( Adults with SSSS often have blood cultures positive for toxigenic[emedicine.com]

Treatment

Upon the diagnosis of SSSS or increased clinical suspicion, beta-lactamase resistant anti-staphylococcal antimicrobials must be promptly administered IV [1].

Therapeutic schemes and dosages include:

  • Newborns: nafcillin 12.5 - 25 mg/kg IV q 6 h (weight should be >2kg)
  • Children: nafcillin 25 to 50 mg/kg 

The schemes above are given until some improvement is observed. Once this has been achieved, administration of the following is required:

  • Infants and children with a weight <20 kg: cloxacillin, 12.5 mg/kg q 6 h per os [12]
  • Older children: cloxacillin 250 to 500 mg q 6 h per os

Vancomycin should be chosen when there is a high suspicion of infection with Methicillin-resistant S. aureus (MRSA). Corticosteroids are strictly contraindicated [1]. Demulcent lotions can also be applied to prevent further dehydration from the ruptured epidermis.

In cases of great severity, with extensive damage, ulcerations and epidermolysis, the treatment followed is the exact same one as for burns (hydrolyzed polymer gel dressings).

Prognosis

The mortality rate of SSSS in children is 4% and mainly depends on the extent of the affected skin [8]. It is easily treated, with the bullae responding well to antimicrobial medications and absence of scarring [9]. 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%.

Etiology

S. aureus is a part of the skin's natural flora, colonizing the nasal cavity, the conjunctival regions and many other regions [2]. 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.

Epidemiology

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 [5].

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.

Sex distribution
Age distribution

Pathophysiology

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 [6]; 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 [7]. 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.

Prevention

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.

Summary

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%) [1].

Patient Information

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.

Causes

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.

Treatment

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.

References

Article

  1. Patel GK, Finlay AY. Staphylococcal scalded skin syndrome: diagnosis and management. Am J Clin Dermatol. 2003; 4(3):165-75.
  2. Ito Y, Funabashi Yoh M, Toda K, et al. Staphylococcal scalded-skin syndrome in an adult due to methicillin-resistant Staphylococcus aureus. J Infect Chemother. 2002 Sep; 8(3):256-61.
  3. Melish ME. Staphylococci, streptococci and the skin: review of impetigo and staphylococcal scalded skin syndrome. Semin Dermatol. 1982; 1:101–109.
  4. McFadden JP, Noble WC, Kemp RD. Superantigenic exotoxin-secreting potential of staphylococci isolated from atopic eczematous skin. Br J Dermatol. 1993; 128:631–632.
  5. Conway DG, Lyon RF, Heiner JD. A desquamating rash; staphylococcal scalded skin syndrome. Ann Emerg Med. 2013 Jan; 61(1):118, 129.
  6. Amagai M, Yamaguchi T, Hanakawa Y, et al. Staphylococcal exfoliative toxin B specifically cleaves desmoglein 1. J Invest Dermatol. 2002 May; 118(5):845-50.
  7. Adhisivam B, Mahadevan S. Abscess of the nasal septum with staphylococcal scalded skin syndrome. Indian Pediatr. 2006 Apr; 43(4):372-3.
  8. Conway DG, Lyon RF, Heiner JD. A desquamating rash; staphylococcal scalded skin syndrome. Ann Emerg Med. 2013 Jan 61(1):118.
  9. Patel NN, Patel DN. Staphylococcal scalded skin syndrome. Am J Med. 2010 Jun; 123(6):505-7.
  10. Ladhani  S, Evans RW. Staphylococcal scalded skin syndrome. Arch Dis Child 1998; 78: 85–88.
  11. Oliveira AR, Aires S, Faria C, et al. Staphylococcal scalded skin syndrome. BMJ Case Rep. 2013 Jun 10; pii: bcr2013009478.
  12. Cribier B, Piemont Y, Grosshans E, et al. Staphylococcal scalded skin syndrome in adults: a clinical review illustrated with a new case. J Am Acad Dermatol.1984; 30:319–324.

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Last updated: 2019-07-11 20:00