Recognizing and Managing Staphylococcal Scalded Skin Syndrome in the Emergency Department.

ABSTRACT: Staphylococcal scalded skin syndrome is a superficial blistering disorder caused by exfoliative toxin-releasing strains of Staphylococcus aureus. Bacterial toxins are released hematogenously, and after a prodromal fever and exquisite tenderness of skin, patients present with tender erythroderma and flaccid bullae with subsequent superficial generalized exfoliation. The head-to-toe directed exfoliation lasts up to 10 to 14 days without scarring after proper treatment. Children younger than 6 years are predominantly affected because of their lack of toxin-neutralizing antibodies and the immature renal system's inability to excrete the causative exotoxins. The epidemiology, pathophysiology, and essential primary skin lesions used to diagnose staphylococcal scalded skin syndrome are summarized for the pediatric emergency medicine physician.

Coagulase-negative staphylococci isolates from blood cultures of newborns in a tertiary hospital in southern Brazil

Abstract Neonatal sepsis continues to be a major cause of morbidity and mortality worldwide. Coagulase-negative staphylococci (CoNS), commonly found on the skin, being the main agents isolated. The aim of this study was to evaluate CoNS isolated from blood cultures of newborn (NB) infants. The study took place between 2014 and 2016/2017 in a tertiary hospital in southern Brazil. Using the VITEK 2 system (bioMérieux, Marcy l'Etoile, France), the microorganisms were identified and had their sensitivity profiles determined. The minimum inhibitory concentrations of linezolid, tigecycline, and vancomycin were also determined. The clinical parameters and mortality rates of NBs were evaluated. From January to December 2014, 176 CoNS isolates were obtained from 131 patients and from June 2016 to July 2017, 120 CoNS isolates were obtained from 79 patients. Staphylococcus epidermidis was most prevalent in both periods. Resistance rates increased between 2014 and 2016/2017, especially against ciprofloxacin (52.27% and 73.11%, p = 0.0004), erythromycin (51.40% and 68.07%, p = 0.0054), gentamicin (50.59% and 67.23%, p = 0.0052), and penicillin (71.3% and 99.17%, p = 0.0001), respectively. With 100% susceptibility to linezolid, tigecycline, and vancomycin in both periods and methodologies tested. In 2014, 53.44% of the NBs received antibiotic therapy, and of these, 77.14% used a catheter; in 2016/2017, these were 78.48% and 95.16%, respectively. Regarding laboratory tests, a hemogram was ineffective, since patients with sepsis presented normal reference values. In 2014 and 2016/17, 15.71% and 17.74% of the NBs died, respectively. S. epidermidis was the predominant microorganism, related to catheter use in most cases. The resistance rates have increased over time, demonstrating the importance of adopting control and prevention measures in this hospital. CoNS are responsible for a significant neonatal sepsis mortality rate in infants.

The spectrum of staphylococcal scalded skin syndrome: a case series in children.

Staphylococcal scalded skin syndrome (SSSS) is a disease caused by certain toxigenic strains of Staphylococcus aureus. While the classic severe phenotype is widely recognized in children, SSSS in fact exists on a spectrum with mild and moderate variants. Misunderstanding the phenotypic spectrum of SSSS may result in misdiagnosis of an otherwise treatable condition. To increase awareness of the heterogeneity of SSSS, we report four cases that together represent a range of clinical presentations.

A young girl with a painful rash.

The speed with which this rash spread and the fact that the patient's skin sloughed off when pressure was applied made the diagnosis clear.

Demographic and clinical characteristics and risk factors for Staphylococcal scalded skin syndrome in Hunan.

OBJECTIVE: To realize the risk factors, clinical features, and treatments of Staphylococcal scalded skin syndrome (SSSS).
 METHODS: The clinical features, laboratory findings, and treatment were retrospectively analyzed in 290 patients from Hunan Children's Hospital.
 RESULTS: Of the 290 patients, less than 3 years old children were 76.6%. One hundred and nine patients had induced factors, and 177 patients had elevated white blood cell count. There were 168 patients with SSSS accompanied with fever, 34 patients accompanied with diarrhea, and 58 patients associated with septicemia. Eighty-five patients performed the bacterial cultures of the skin secretions, 21 did the throat swab, and 13 did both of the skin secretions and throat swab. Bacterial culture results showed that 119 samples were positive for Staphylococci. All patients were cured after antimicrobial therapy. The skin lesions were improved in 3.26 d. The mean hospital stay was 6.55 d. Recovery time of the body temperature was 3.48 d in average.
 CONCLUSION: SSSS predominates in infants and children under 3 years old, and has tendency to combine with multi-organ symptoms. The early diagnosis and active antimicrobial treatment are the keys of successful treatments.

Use of the frozen section 'jelly-roll' technique to aid in the diagnosis of bullous congenital ichthyosiform erythroderma (epidermolytic hyperkeratosis).

Frozen section is a valuable tool that is often underutilized in the setting of in-patient dermatology. Traditionally, frozen section has been used in dermatology to diagnose toxic epidermal necrolysis, with some additional utility in staphylococcal scalded skin syndrome in the new born period. We report a newborn female with ruptured bullae on the face, chest, back and extremities with a clinical differential diagnosis that included staphylococcal scalded skin, bullous congenital ichthyosiform erythroderma/epidermolytic hyperkeratosis and epidermolysis bullosa. A thin detached skin sample ('jelly-roll') taken from a ruptured bulla on the abdomen was prepared for frozen section analysis. Characteristic findings of epidermolytic hyperkeratosis were seen which included hyperkeratosis with granular layer degeneration, vacuolization and eosinophilic globules. The 'jelly-roll' technique can be used for quick diagnosis with minimal trauma to the patient. Epidermolytic hyperkeratosis was subsequently confirmed by a biopsy fixed in formalin and by genetic testing. A novel missense mutation in KRT1 (I479N) was identified. Herein, we discuss the use of the frozen section 'jelly roll' technique for rapid diagnosis in a case of bullous congenital ichthyosis erythroderma/epidermolytic hyperkeratosis.

Skin biopsy: Biopsy issues in specific diseases.

Misdiagnosis may result from biopsy site selection, technique, or choice of transport media. Important potential sources of error include false-negative direct immunofluorescence results based on poor site selection, uninformative biopsy specimens based on both site selection and technique, and spurious interpretations of pigmented lesions and nonmelanoma skin cancer based on biopsy technique. Part I of this 2-part continuing medical education article addresses common pitfalls involving site selection and biopsy technique in the diagnosis of bullous diseases, vasculitis, panniculitis, connective tissue diseases, drug eruptions, graft-versus-host disease, staphylococcal scalded skin syndrome, hair disorders, and neoplastic disorders. Understanding these potential pitfalls can result in improved diagnostic yield and patient outcomes.

A case report of differentiating staphylococcal scalded skin syndrome and toxic epidermal necrolysis by optical coherence tomography.

BACKGROUND: Staphylococcal scalded skin syndrome (SSSS) and toxic epidermal necrolysis (TEN) both present with acute onset, high morbidity and significant mortality. Rapid diagnosis is therefore of importance. The aim of this study was to investigate and compare the presentation of these diseases using optical coherence tomography (OCT). METHODS: Two male patients with bullous diseases, SSSS and TEN, respectively, were photographed digitally, examined using dermoscopy, OCT scanned and subsequently biopsied in the said order. RESULTS: The bullous skin was visualized by OCT showing two distinct images: the SSSS-patient displayed superficial hyporefletive flaccid structures with a split high in the thickened (0.51 mm vs. 0.12 mm) epidermis while the TEN-patient demonstrated a larger hyporeflective ovoid structure with a split right below the thickened epidermis (0.18 mm vs. 0.06 mm). CONCLUSION: These findings suggest that there is a potential for the application of OCT scanning in the acute phase of SSSS and TEN in order to distinguish them for a faster diagnosis and better management and treatment.

From erythema multiforme to toxic epidermal necrolysis. Same spectrum or different diseases?

Erythema multiforme (EM), Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute bullous disorders associated to different prognosis, mainly due to infections and drugs. More in particular EM in more than 90% is caused by infections (especially Herpes virus infection), while, on the other hand SJS and TEN are referable in more than 95% of cases to drugs. Distinction among these three forms is often controversal and still debated. An attempt to distinguish these forms has been possible mainly according to anamnesis, clinical presentation (morphology, involved sites, extension of lesions) and pathogenetic mechanisms, being on the contrary more difficult from an histopathological point of view. Nowadays a clear diagnosis and a distinction from other life-threatening diseases is possible with the integration of all the mentioned aspects. Moreover, this recognition should be as early as possible in order to perform a prognostic evaluation of the case and to start supportive cares and therapies as soon as possible.

[Staphylococcal scalded skin syndrome associated with long-term catherter related infection in an adult].

Staphylococcal scalded skin syndrome (SSSS) is an extensive desquamative erythmatous condition caused by the Staphylococcus aureus exfoliative toxin. Although adult cases of SSSS are rare, the mortality rate is high. We report herein on a case of SSSS due to long-term catheter-related bloodstream infection caused by exfoliative toxin B, which produced methicillin-resistant Staphylococcus aureus. A 64-year-old man was admitted to our hospital with a high fever and generalized exfoliative dermatitis. He had an implanted port vascular access device in his left arm. The port was removed because it was thought to be the focus of infection. A Gram stain of the pus from the incision site revealed Gram positive coccus in clusters, and we administered intravenous vancomycin. MRSA was isolated from blood cultures and the pus, and histiology of a skin biopsy specimen from the exfoliation dermatitis showed epidermal detachment in the uppermost layer, which was consistent with SSSS. Although the patient developed infective endocarditis and septic embolisms, he eventually recovered. PCR of the MRSA was positive for exfoliative toxin B, and we finally diagnosed an adult case of SSSS due to exfoliative toxin B producing MRSA.

Skin findings of Staphylococcus aureus toxin-mediated infection in relation to toxin encoding genes.

BACKGROUND: Staphylococcal scalded skin syndrome and toxic shock syndrome are associated with exfoliatins and superantigens, respectively; and are easy to distinguish in their usual presentation. However, there is confusion about the mild forms of these 2 staphylococcal diseases. These mild forms are both designated as "staphylococcal scarlet fever" despite differences in their pathophysiology and clinical presentation. Our study aimed to distinguish between the clinical characteristics of the rash associated with exfoliatins and the rash associated with superantigens. METHODS: Patients were selected from the French National Reference Center for Staphylococci. We retrospectively compared the clinical characteristics of patients with a generalized rash during Staphylococcus aureus infection. Patients who met the criteria of staphylococcal scalded skin syndrome or toxic shock syndrome were excluded. The patients were classified into 2 groups depending on the presence of a gene coding for exfoliatin or for superantigenic toxin. RESULTS: We included 13 cases with exfoliatin and 9 with superantigens. The patients of the exfoliatin group were more likely to have facial involvement, fold involvement and a superficial focus of infection. In the second group, S. aureus was isolated from a deeper focus in 8 of 9 patients. CONCLUSION: Mild forms of S. aureus toxin-mediated infection affect the pediatric population. Examination made it possible to distinguish an exanthema associated with an exfoliatin from one associated with a superantigen. This early clinical distinction results in differences in management.

[Acute skin detachment in a newborn]. / Acute huidloslating bij een pasgeborene.

Acute blistering and erosion in a newborn is one of the few emergency cases seen in dermatology. It is important to differentiate between infectious causes, congenital abnormalities, autoimmune bullous dermatitis, immunological skin diseases and skin burns within 24 hours. In this clinical lesson, we present a case of acute skin detachment in a newborn caused by staphylococcal scalded skin syndrome (SSSS). Our patient was a six-day-old boy who had developed flaccid blisters around the umbilicus, which ruptured on minimal friction. Generalised superficial erosions on the face, hands and feet arose within hours. Based on the clinical presentation combined with a subcorneal blister found on histopathological examination and a positive culture for Staphylococcus aureus on nasal and umbilical smears, the diagnosis of SSSS was made. Our patient was treated successfully with flucloxacillin and gentamicin; the skin lesions healed without scarring within six days.

Varicella infection in a neonate with subsequent staphylococcal scalded skin syndrome and fatal shock.

A male term neonate, at day 23 of life, presented with vesicular lesions over the trunk, which spread to allover the body on the next day. Five days later, he started developing blistering of the skin over the trunk and extremities, which subsequently ruptured, leaving erythematous, tender raw areas with peeling of the skin. The mother had vesicular eruptions, which started on the second day of delivery and progressed over the next 3 days. Subsequently, similar eruptions were noticed in two of the siblings before affecting the neonate. On the basis of the exposure history and clinical picture, a diagnosis was made of varicella infection with staphylococcal scalded skin syndrome (SSSS). The blood culture and the wound surface culture grew Staphylococcus aureus. Treatment included intravenous fluid, antibiotics, acyclovir and wound care. However, after 72 h of hospitalisation, the neonate first developed shock, refractory to fluid boluses, vasopressors and catecholamine along with other supports; and he then succumbed. In all neonates, staphylococcal infection with varicella can be fatal due to SSSS, the toxic shock syndrome or septicaemia.

Staphylococcal scalded skin syndrome after intra-articular injection of hyaluronic acid.

One of the severe adverse effects of intra-articular injection in the knee is septic arthritis of the knee joint. Staphylococcus aureus is the most frequent pathogen of septic arthritis. Staphylococcal scalded skin syndrome (SSSS) refers to a spectrum of blistering skin diseases caused by S. aureus exfoliative toxins. Although SSSS is rarely observed in adults, the mortality rate is high in adult cases. We report a case of SSSS due to septic knee arthritis after intra-articular hyaluronic acid injections.