Epidermolytic ichthyosis complicated by staphylococcal scalded skin syndrome in the newborn.

Epidermolytic ichthyosis is characterized by erythema and blistering at birth. We present a neonate with epidermolytic ichthyosis who had a subtle change in clinical findings while hospitalized, including increased fussiness, erythema, and a change in her skin odor, which represented superimposed staphylococcal scalded skin syndrome. This case highlights the unique challenge of recognizing cutaneous infections in neonates with blistering skin disorders and emphasizes the importance of having a high suspicion for superinfection in this population.

Staphylococcal scalded skin syndrome: An epidemiological and clinical review of 84 cases.

BACKGROUND: Staphylococcal scalded skin syndrome (SSSS) is a toxin-mediated, blistering skin disorder that mainly affects infants and children. There is limited literature regarding pediatric SSSS. The purpose of this study was to describe the epidemiology, clinical features, and management of pediatric SSSS. METHODS: Retrospective cohort study of pediatric patients with a clinical diagnosis of SSSS seen at the Hospital for Sick Children in Toronto, Ontario, Canada, from January 1994 to March 2016. RESULTS: We included 84 patients with a clinical diagnosis of SSSS; 49/84 (58%) were male. Mean age of diagnosis was 3.1 ± 2.4 years. All patients presented with erythema and exfoliation, while 64/84 (76%) presented with vesicles/ bullae. Skin tenderness was the most common symptom, present in 68/84 (81%) subjects. Staphylococcus aureus was more commonly isolated from periorificial cultures than from bullae. Mean hospitalization was 4.7 ± 2.3 days. No difference was found in admission duration between children receiving clindamycin and those that did not (3.6 ± 2.2 vs 3.9 ± 2.34 days, P = .63). Skin debridement was the only risk factor leading to more complications and prolonged hospitalization (P = .03). Severe complications were seen in 4 (5%) cases, and no fatalities were observed. CONCLUSIONS: Healthcare providers should be aware of SSSS and consider it in the differential diagnosis of infants and children with new onset erythema, exfoliation, and/or vesiculation. Suspected culprit pathogens were more often obtained from periorificial swabs; however, these isolates were not tested for exfoliative toxin to confirm causality. Antibiotic treatment should be guided by sensitivity testing. Addition of clindamycin as an anti-toxin agent had no effect on the duration of hospitalization, and this should be further investigated. Surgical debridement of the skin in patients with SSSS should be discouraged.

Staphylococcal scalded skin syndrome in neonates: an 8-year retrospective study in a single institution.

Staphylococcal scalded skin syndrome (SSSS) is a rare disorder in children. Complications may occur without timely treatment. Mortality in children with SSSS is approximately 4%. Other than a limited number of case reports, data on SSSS in neonates are limited. The objective of the current study was to investigate SSSS in neonates. A retrospective review of neonates with a diagnosis of SSSS from January 2004 to January 2012 was performed. Population distribution, historical features, physical examination findings including laboratory tests, antibiotic therapies, and outcomes were evaluated. Thirty-nine cases were included, 31 (79.5%) in the last 4 years. The mean patient age was 17.4 ± 7.7 days. Boys (25 cases) were more commonly affected, and occurrence during summer and autumn months was more frequent. The face was the most common body part affected and the area most commonly initially affected. Fever, high white blood cell count, and high C-reactive protein levels were uncommon. Pneumonia was the most frequent complication (74.4%). The positive rate of Staphylococcus aureus isolation was low (23.5%). Drug susceptibility tests showed that amoxicillin with clavulanic acid and cephalosporins were effective in practice. The median length of hospitalization was 9.0 days. All of the 39 neonates were cured without scarring. This study established basic epidemiologic characteristics of a group of neonates diagnosed with SSSS. In the presence of a clinical suspicion of SSSS, even with apparently normal laboratory tests, immediate treatment with cephalosporins, ß-lactamase-resistant semisynthetic penicillin, or both is advocated.

Nosocomial outbreak of staphyloccocal scalded skin syndrome in neonates in England, December 2012 to March 2013.

Staphylococcal scalded skin syndrome (SSSS) is a blistering skin condition caused by exfoliative toxin-producing strains of Staphylococcus aureus. Outbreaks of SSSS in maternity settings are rarely reported. We describe an outbreak of SSSS that occurred among neonates born at a maternity unit in England during December 2012 to March 2013. Detailed epidemiological and microbiological investigations were undertaken. Eight neonates were found to be infected with the outbreak strain of S. aureus, of spa type t346, representing a single pulsotype. All eight isolates contained genes encoding exfoliative toxin A (eta) and six of them contained genes encoding toxin B (etb). Nasal swabs taken during targeted staff screening yielded a staphylococcal carriage rate of 21% (17/80), but none contained the outbreak strain. Mass screening involving multi-site swabbing and pooled, enrichment culture identified a healthcare worker (HCW) with the outbreak strain. This HCW was known to have a chronic skin condition and their initial nasal screen was negative. The outbreak ended when they were excluded from work. This outbreak highlights the need for implementing robust swabbing and culture methodswhen conventional techniques are unsuccessful in identifying staff carrier(s). This study adds to the growing body of evidence on the role of HCWs in nosocomial transmission of S. aureus.

Neonatal staphylococcal scalded skin syndrome: clinical and outbreak containment review.

Staphylococcal scalded skin syndrome (SSSS) is a toxin-mediated exfoliating skin condition predominated by desquamation and blistering. Neonatal outbreaks have already been reported; however, our outbreak highlights the potential for SSSS following neonatal health promotion measures such as intra-muscular vitamin K administration and metabolic screening (heel prick) as well as effective case containment measures and the value of staff screening. Between February and June 2007, five confirmed cases of neonatal SSSS were identified in full-term neonates born in an Irish regional maternity hospital. All infants were treated successfully. Analysis of contact and environmental screening was undertaken, including family members and healthcare workers. Molecular typing on isolates was carried out. An outbreak control team (OCT) was assembled and took successful prospective steps to prevent further cases. All five Staphylococcus aureus isolates tested positive for exfoliative toxin A, of which two distinct strains were identified on pulsed-field gel electrophoresis analysis. Two cases followed staphylococcal inoculation during preventive measures such as intra-muscular vitamin K administration and metabolic screening (heel prick). None of the neonatal isolates were methicillin resistant. Of 259 hospital staff (70% of staff) screened, 30% were colonised with S. aureus, and 6% were positive for MRSA carriage. This is the first reported outbreak of neonatal SSSS in Ireland. Effective case containment measures and clinical value of OCT is demonstrated. Results of staff screening underlines the need for vigilance and compliance in hand disinfection strategies in maternity hospitals especially during neonatal screening and preventive procedures.

Increasing Numbers of Staphylococcal Scalded Skin Syndrome Cases Caused by ST121 in Houston, Texas.

BACKGROUND: The molecular epidemiology of Staphylococcus aureus strains causing staphylococcal scalded skin syndrome (SSSS) in the United States has not been described. We analyzed patient and S. aureus isolate characteristics associated with SSSS in children at Texas Children's Hospital. METHODS: Patients with SSSS were identified by ICD9/10 codes and available S. aureus isolates were identified from an ongoing S. aureus surveillance study. Medical records were reviewed for 58 patients with available S. aureus isolates. Isolate analyses included PCR for agr group, pvl (lukSF-PV), tst, eta and etb, pulsed-field gel electrophoresis, multi-locus sequence typing and antimicrobial susceptibilities. RESULTS: Cases of SSSS increased from 2.3/10,000 admissions in 2008 to 52.6/10,000 admissions in 2017 (P < 0.0001). The 58 study cases (57 methicillin-susceptible S. aureus, 1 MRSA) with isolates were from 2013 to 2017. The majority (88%) of isolates was of clonal cluster (CC) 121, agr group IV, pvl, tst and carried eta and/or etb and 26% were clindamycin resistant. Twelve ST121 isolates had high level resistance to mupirocin. Patients were treated with standard supportive care plus systemic antibiotics [clindamycin alone or in combination with another antibiotic (n = 44)]. One patient had a recurrent SSSS and one patient was transferred to a burn unit on day 3. CONCLUSIONS: Cases of SSSS are increasing at our hospital. Most S. aureus strains isolated were of one CC, CC121 and carried eta and etb. Supportive care plus clindamycin was effective treatment. We speculate that CC121 was recently introduced to our region and is responsible for the increasing numbers of SSSS cases observed at Texas Children's Hospital.

Staphylococcal-scalded skin syndrome: evaluation, diagnosis, and management.

BACKGROUND: Staphylococcal-scalded skin syndrome (SSSS), also known as Ritter disease, is a potentially life-threatening disorder and a pediatric emergency. Early diagnosis and treatment is imperative to reduce the morbidity and mortality of this condition. The purpose of this article is to familiarize physicians with the evaluation, diagnosis, and treatment of SSSS. DATA SOURCES: A PubMed search was completed in Clinical Queries using the key terms "Staphylococcal scalded skin syndrome" and "Ritter disease". RESULTS: SSSS is caused by toxigenic strains of Staphylococcus aureus. Hydrolysis of the amino-terminal extracellular domain of desmoglein 1 by staphylococcal exfoliative toxins results in disruption of keratinocytes adhesion and cleavage within the stratum granulosum which leads to bulla formation. The diagnosis is mainly clinical, based on the findings of tender erythroderma, bullae, and desquamation with a scalded appearance especially in friction zones, periorificial scabs/crusting, positive Nikolsky sign, and absence of mucosal involvement. Prompt empiric treatment with intravenous anti-staphylococcal antibiotic such as nafcillin, oxacillin, or flucloxacillin is essential until cultures are available to guide therapy. Clarithromycin or cefuroxime may be used should the patient have penicillin allergy. If the patient is not improving, critically ill, or in communities where the prevalence of methicillin-resistant S. aureus is high, vancomycin should be used. CONCLUSION: A high index of suspicion is essential for an accurate diagnosis to be made and treatment promptly initiated.

Rapid containment of nosocomial transmission of a rare community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) clone, responsible for the Staphylococcal Scalded Skin Syndrome (SSSS).

BACKGROUND: The aims of this study were to identify the source and the transmission pathway for a Staphylococcal Scalded Skin Syndrome (SSSS) outbreak in a maternity setting in Italy over 2 months, during 2014; to implement appropriate control measures in order to prevent the epidemic spread within the maternity ward; and to identify the Methicillin-Resistant Staphylococcus aureus (MRSA) epidemic clone. METHODS: Epidemiological and microbiological investigations, based on phenotyping and genotyping methods, were performed. All neonates involved in the outbreak underwent clinical and microbiological investigations to detect the cause of illness. Parents and healthcare workers were screened for Staphylococcus aureus to identify asymptomatic carriers. RESULTS: The SSSS outbreak was due to the cross-transmission of a rare clone of ST5-CA-MRSA-SCCmecV-spa type t311, exfoliative toxin A-producer, isolated from three neonates, one mother (from her nose and from dermatological lesions due to pre-existing hand eczema) and from a nurse (colonized in her nose by this microorganism). The epidemiological and microbiological investigation confirmed these as two potential carriers. CONCLUSIONS: A rapid containment of these infections was obtained only after implementation of robust swabbing of mothers and healthcare workers. The use of molecular methodologies for typing was able to identify all carriers and to trace the transmission.

Nosocomial outbreak of staphylococcal scalded skin syndrome in neonates: epidemiological investigation and control.

Over a three-month period, 13 neonates developed staphylococcal scalded skin syndrome (SSSS) in a maternity unit, between four and 18 days after their birth. An epidemiological and descriptive study followed by a case-control study was performed. A case was defined as a neonate with blistering or peeling skin, and exfoliative toxin A Staphylococcus aureus positive cultures. Controls were selected at random from the asymptomatic, non-colonized neonates born on the same day as the cases. All staff members and all neonates born during the outbreak period were screened for carriage by nasal swabs and umbilical swabs, respectively. S. aureus isolates were polymerase chain reaction (PCR) screened for etA gene and genotyped by pulsed-field gel electrophoresis (PFGE). Two clusters of eight and five cases were identified. Receiving more than one early umbilical care procedure by the same ancillary nurse was the only risk factor identified in the case-control study (odds ratio=15, 95% confidence intervals 2-328). The ancillary nurse suffered from chronic dermatitis on her hands that favoured S. aureus carriage. Exfoliative-toxin-A-producing strains, as evidenced by PCR and indistinguishable by PFGE, were isolated from all but one of the SSSS cases, from four asymptomatic neonates, from two staff members and from the ancillary nurse's hands. Removal of the ancillary nurse from duty, infection control measures (isolation precautions, chlorhexidine handwashing and barrier protections), and treatment of the carriers (nasal mupirocin and chlorhexidine showers) led to control of the epidemic. In conclusion, this study emphasizes the need for tight surveillance of chronic dermatitis in healthcare workers.

Molecular epidemiology of staphylococcal scalded skin syndrome in premature infants.

BACKGROUND: Outbreaks of nosocomial staphylococcal scalded skin syndrome (SSSS) in infants have been well-described associated with the well baby nursery or delivery room. We describe two cases of SSSS in very low birth weight infants in a neonatal intensive care unit (NICU) and the success of infection control strategies used to prevent an outbreak. METHODS: Staphylococcal scalded skin syndrome was diagnosed in two infants in the NICU: Case I (a 47-day-old, formerly 530-g female); and Case II diagnosed 48 h later (a 41-day old, formerly 706-g female). Multiple infection control measures were implemented: (1) isolation and intravenous antibiotic treatment of cases; (2) placement of exposed infants into a cohort; (3) prophylactic mupirocin treatment of the anterior nares of all infants in the NICU and staff colonized with Staphylococcus aureus; and (4) personnel hand washing with hexachlorophene. Detection of exfoliative toxin A and studies to determine the genetic relatedness of S. aureus strains isolated from patients and staff were performed. RESULTS: In addition to the two SSSS cases, S. aureus was isolated from 2 of 12 (17%) exposed asymptomatic infants, 2 of 20 (10%) ancillary staff, 8 of 30 (27%) nurses and 6 of 24 (25%) physicians. Exfoliative toxin A-producing strains were isolated from both cases and one asymptomatic infant. No toxin was expressed by strains isolated from staff. Pulse field gel electrophoresis demonstrated genetically identical strains of S. aureus from the two SSSS cases and the asymptomatic infant, whereas three staff members harbored strains genetically related to the case strain. Unexpectedly two additional unique clusters of genetically related S. aureus strains were identified from the surveillance cultures. CONCLUSIONS: This report documents the rare occurrence of nosocomial SSSS attributed to transmission in the NICU among extremely low birth weight infants. Multiple infection control strategies were effective in limiting the outbreak. Molecular epidemiology investigation supported a unique S. aureus strain responsible for this event and the presence of bidirectional spread between staff and patients of non-toxin-producing strains.

Staphylococcal scalded skin syndrome.

Staphylococcal scalded skin syndrome (SSSS) is a recognised clinical entity that affects primarily the very young and, in rare cases, the very old or the immunocompromised. Koch's postulates have been fulfilled in that: (i) Staphylococcus aureus is isolated from every case; (ii) S. aureus can reproduce the syndrome in an experimental animal model; (iii) a specific extracellular toxin can reproduce the syndrome; and (iv) antibody to the toxin can protect experimental animals. Although exfoliative toxin (ET) is responsible for the skin loosening seen in SSSS, it does not account for all the symptoms of the disease. Purified ET does not cause erythema in either neonatal mice or man, and the lesions are not painful unless the loosened epidermis is removed. This suggests that other factors, e.g., delta-haemolysin, are involved in the pathogenesis of this condition. Although much has been learned about the pathogenesis of the syndrome, we are still largely ignorant of the factors which govern host resistance to SSSS (i.e., intoxication by ET-producing strains of S. aureus). It is fortunate from the patient's point of view that the aetiological agent can be destroyed readily by the use of appropriate antibiotic therapy.

Staphylococcal scalded skin syndrome: diagnosis and management.

Staphylococcal scalded skin syndrome (SSSS) is a common disorder that is usually seen in infants and children and rarely seen in adults. SSSS usually presents with a prodrome of sore throat or conjunctivitis. Extremely tender flaccid bullae, which are Nikolsky sign-positive, develop within 48 hours and commonly affect the flexures; occasionally, large areas of the skin may be involved. The bullae enlarge and rupture easily to reveal a moist erythematous base, which gives rise to the scalded appearance. SSSS in adults is a rare disorder, though there are now over 50 documented cases. Usually SSSS occurs in predisposed individuals, but not all adults have an underlying illness. Whereas mortality in childhood SSSS is approximately 4%, the mortality rate in adults is reported to be greater than 60%. SSSS is caused by an infection with a particular strain of Staphylococcus aureus, which leads to blistering of the upper layer of the skin, by the release of a circulating exotoxin. It has recently been demonstrated that the exfoliative exotoxin responsible for SSSS leads to the cleavage of desmoglein 1 complex, an important desmosomal protein. The same toxins that are responsible for causing SSSS also cause bullous impetigo. There appears to be a relationship between the disease extent, the amount of toxin produced and whether the toxin is released locally or systemically. As a result there is likely to be a spectrum of disease and there are likely to be a number of milder cases of adult SSSS that go undiagnosed. Social improvements and hygiene have led to a dramatic fall in the number of cases of SSSS. Treatment is usually straightforward, when there is no coexistent morbidity and the presentation is mild, but can be demanding if the patient is particularly ill. SSSS is still associated with mortality, particularly when it occurs in adults.

Treatment of bullous impetigo and the staphylococcal scalded skin syndrome in infants.

Impetigo is a common, superficial, bacterial infection of the skin characterized by an inflamed and infected epidermis. The rarer variant, bullous impetigo, is characterized by fragile fluid-filled vesicles and flaccid blisters and is invariably caused by pathogenic strains of Staphylococcus aureus. Bullous impetigo is at the mild end of a spectrum of blistering skin diseases caused by a staphylococcal exfoliative toxin that, at the other extreme, is represented by widespread painful blistering and superficial denudation (the staphylococcal scalded skin syndrome). In bullous impetigo, the exfoliative toxins are restricted to the area of infection, and bacteria can be cultured from the blister contents. In staphylococcal scalded skin syndrome the exfoliative toxins are spread hematogenously from a localized source causing widespread epidermal damage at distant sites. Both occur more commonly in children under 5 years of age and particularly in neonates. It is important to swab the skin for bacteriological confirmation and antibiotic sensitivities and, in the case of staphylococcal scalded skin syndrome, to identify the primary focus of infection. Topical therapy should constitute either fusidic acid (Fucidin, Leo Pharma Ltd) as a first-line treatment, or mupirocin (Bactroban, GlaxoSmithKline) in proven cases of bacterial resistance. First-line systemic therapy is oral or intravenous flucloxacillin (Floxapen, GlaxoSmithKline). Nasal swabs from the patient and immediate relatives should be performed to identify asymptomatic nasal carriers of Staphylococcus aureus. In the case of outbreaks on wards and in nurseries, healthcare professionals should also be swabbed.

Outbreak of staphylococcal scalded skin syndrome among neonates.

Over a period of 2 months, 12 babies born in the maternity unit at Guy's Hospital developed staphylococcal scalded skin syndrome in two distinct outbreaks. Staphylococci isolated from the babies, together with those from the mothers and attending medical staff were phage-typed. All isolates from the babies were of type 3A/3C. During the first outbreak only one carrier of the epidemic strain (a paediatrician) was found but a further 12 persons were identified as possible carriers during the second outbreak. In order to confirm the link between outbreaks, all phage group II isolates were subjected to reverse phage-typing, testing for metal-ion resistance, plasmid profiling and in-vivo testing for production of epidermolytic toxin. It was shown that the same epidemic strain of toxin-producing Staphylococcus aureus was responsible for both outbreaks. The affected neonates responded rapidly to a short course of intravenous flucloxacillin. The outbreak ceased after appropriate treatment of all carriers and the implementation of an extensive disinfection policy within the maternity unit.

Staphylococcal scalded skin syndrome in the Czech Republic: an epidemiological study.

OBJECTIVE: To identify the basic epidemiological characteristics of children hospitalized with diagnosis of Staphylococcal scalded skin syndrome in the Czech Republic in the years 1994-2009. INTRODUCTION: Staphylococcal scalded skin syndrome (SSSS) is a relatively rare disease in childhood. This syndrome was first defined in 1878 by Baron Gottfried Ritter von Rittershainem and belongs to the group of diseases called Burn-like syndromes. It is a bullous skin disease caused by exfoliative toxins which are produced by certain types of Staphyloccocus aureus. Typical structures affected by these toxins are desmosome proteins called Desmoglein-1 located in the stratum granulosum of epidermis. Unlike in Lyell's syndrome or Stevens-Johnson's syndrome, the exfoliation is caused by loss of adhesivity particularly in the stratum granulosum and not by induction of apoptosis in the dermo-epidermal junction. MATERIAL AND METHODS: This retrospective study was conducted on patients hospitalized in the Czech Republic in the period from 1.1.1994 to 31.12.2009. The basic condition for the inclusion in the retrospective study was age under 1 year and hospitalization due to SSSS. A total of 399 children (177 girls) fulfilled the criteria for inclusion into the study. Information was obtained from a central data depository, the Department of Health Information and Statistics, Czech Republic. RESULTS: A total of 399 children under 1 year were hospitalized for the diagnosis of SSSS in the study period. The group included 177 girls and 222 boys. M:F ratio was 1.25:1. The average incidence of SSSS in the Czech Republic was 25.11 cases per 100,000 children under 1 year of age. The highest recorded incidence in the followed period was in 1994, when a total of 57 cases of SSSS was reported, namely 53.47 per 100,000 children. By contrast, in 2003, there were reported only 12 cases and the incidence of 12.81 per 100,000 children. The average length of hospitalization was 6.39 days. In 1995, the highest average length of hospitalization was reported, which was 8.1 days, and then in 2007, the lowest average length of hospitalization, 4.4 days. There was no significant difference in the length of hospitalization in boys and girls. None of the 399 children in the population died. CONCLUSION: In our retrospective study, we established basic epidemiological characteristics of a group of children aged under 1 year with diagnosis of SSSS. As epidemiological data show, the occurrence of this syndrome is not sporadic, but steady.

Epidemiological data of staphylococcal scalded skin syndrome in France from 1997 to 2007 and microbiological characteristics of Staphylococcus aureus associated strains.

Epidemiological data on staphylococcal scalded skin syndromes (SSSS), including bullous impetigo (BI) and generalized exfoliative syndrome (GES), are scarce. To better characterize SSSS and associated Staphylococcus aureus strains, we conducted a retrospective study of 349 cases collected in France between 1997 and 2007 by the National Reference Centre of Staphylococci. Our results showed a stationary evolution of SSSS cases, with a heterogeneous distribution of cases in France. Although notification was not exhaustive, we estimated an incidence of 0.56 cases/year/million inhabitants, in accordance with previous studies conducted in France and Europe, with a median age of 2 years old and sex ratios of 1. A seasonal effect was observed, with a higher GES/BI ratio in autumn compared with other seasons, which could be explained by the impact of viral co-infection. Genetic analysis of S. aureus strains showed that accessory gene regulator (agr) 4, exfoliative toxin A (eta) and B (etb) genes, staphylococcal and enterotoxin-like O (selo) gene and agr4 etb selo profiles were predominantly associated with GES, whereas agr2 eta and agr4 eta selo were more frequently observed with BI. Only one methicillin-resistant strain was found. Protein A (spa) typing identified two main genotypes: spa clonal complex (CC) 159/sequence-type (ST) 121 (75%) and spaCC346/ST15 (18%). spaCC159 was mainly associated with agr4 eta etb selo, agr4 eta selo and agr4 etb selo, and spaCC346 was mainly associated with agr2 eta, suggesting that French SSSS cases are caused by these two main lineages. However, in a multivariate analysis, only etb was independently associated with GES.