Presentation and Management of Staphylococcal Scalded Skin Syndrome in a Child After a Burn Injury: A Case Report.

This case report describes the clinical course of a child who developed staphylococcal scalded skin syndrome (SSSS) after a burn injury. The intent is to aid other units in recognizing the presentation of SSSS after a pediatric burn and to optimize subsequent management. The main clinical finding was of rapid, progressive, superficial epidermal loss at sites separate from the original burn, involving 55% of the total body surface area, 13 days after a 6% scald burn to the face, neck, and chest. Diagnosis was confirmed by multidisciplinary team clinical assessment and histopathology of an intraoperative skin biopsy. This confirmed epidermal cleavage at the granular cell layer. These findings were later supported by Staphylococcus aureus cultured from the burn wound, and a positive epidermolytic toxin A assay. Management was with general medical supportive care, clindamycin and flucloxacillin intravenous antibiotic therapy, and cleansing and dressing of the areas of epidermal loss. Key learning points from this case were that SSSS presented after a burn injury and that 13 days elapsed between the burn and SSSS. Factors differentiating it from toxic epidermal necrolysis are described, including the value of histopathology in confirming the diagnosis. The prompt use of antibiotics and attentive wound care are advocated as an effective management strategy.

Intérêt de la synéchiolyse pour prévenir le symblépharon au cours des épidermolyses toxiques : à propos de deux observations au complexe pédiatrique de Bangui (République Centrafricaine)

Le syndrome de Stevens-Johnson et le syndrome de Lyell, ou nécrolyse épidermique toxique, représentent des réactions d'hypersensibilité cutanées rares, habituellement d'origine médicamenteuse, potentiellement mortelles. Les atteintes ophtalmologiques au cours de ces syndromes peuvent être graves et conduire à la cécité. Ces atteintes peuvent être prises en charge par des soins oculaires appropriés. Nous rapportons deux cas de syndrome de Lyell: le premier ayant présenté une complication oculaire due à l'absence de la prise en charge oculaire en phase aigüe. Le deuxième ayant bénéficié de la technique de synéchiolyse

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.

Cleavage isn't everything: potential novel mechanisms of exfoliative toxin-mediated blistering.

This Commentary describes breakthroughs in understanding the interactions between desmoglein 1 and plakogloben in staphylococcal-mediated blistering skin diseases.

Síndrome de escaldadura estafilocócica / Staphylococcal scalded skin syndrome

Introducción: El síndrome de escaldadura estafilocócica es una enfermedad causada por Staphylococcus aureus productor de toxinas exfoliativas. Es poco frecuente y apenas hay datos de su epidemiología en nuestro país. Pacientes y métodos: Se llevó a cabo un estudio observacional de cohortes en el que se revisaron los casos de escaldadura estafilocócica controlados en el Hospital Materno-Infantil La Paz en los últimos 10 años (enero 1997-diciembre 2006). Resultados: Se obtuvo un total de 26 pacientes, 7 en los primeros 5 años y 19 en los siguientes, con una media de edad en el momento del diagnóstico de 29 meses; 4 casos (15 %) fueron de diagnóstico neonatal. El 67 % de los casos ocurrieron en primavera y verano. Las manifestaciones clínicas más frecuentes fueron: eritrodermia con aparición de ampollas y descamación posterior (100 %), fisuración y costras periorales (54 %), fiebre (46 %), conjuntivitis (42 %) y edema palpebral (31 %). En la analítica no se detectó un aumento significativo de leucocitos (media: 11.341/μl) ni de proteína C reactiva (media: 9 mg/l). El diagnóstico fue principalmente clínico, con aislamiento de S. aureus en frotis nasal o conjuntival en el 59 % de los casos. Todas las cepas fueron sensibles a cloxacilina, clindamicina y vancomicina. El tratamiento se realizó con cloxacilina intravenosa con evolución favorable. Conclusiones: El síndrome de escaldadura estafilocócica parece ser más frecuente en los últimos años y debe sospecharse en un niño con eritrodermia aguda y afectación conjuntival o peribucal. El tratamiento con cloxacilina consigue la curación sin secuelas (AU)

Introduction: Staphylococcal scalded skin syndrome is a rare disease caused by Staphylococcus aureus that produces exfoliative toxins. There are few epidemiological data in our environment. Patients and methods: We present an observational cohort study. We review the cases of staphylococcal scalded skin syndrome monitored at La Paz Children Hospital during the last ten years (January 1997 to December 2006). Results: We obtained 26 patients, 7 in the first 5 years and 19 more in the following years. The mean age at diagnosis was 19 months. Four cases (15 %) occurred during the neonatal period. Sixty-seven percent of the cases were diagnosed during spring and summer. Main clinical signs were: erythroderma with blisters and posterior desquamation (100 %), perioral fissures (54 %), fever (46 %), conjunctivitis (42 %) and palpebral edema (31 %). No significant increases in leukocytes (mean: 11,341/μl) or C-reactive proein (mean: 9 mg/l) were found on blood analysis. Diagnosis was made by clinical findings. S. aureus was isolated in nasal or conjunctival samples on 59 % of cases. All strains were sensitive to cloxacillin, clindamycin and vancomycin. The patients were treated with cloxacillin with good progress. Conclusions: Staphylococcal scalded skin syndrome seems to be more common in the last few years. It must be suspected in children with acute erythroderma and perioral or conjunctival lesions. Treatment with cloxacillin leads to healing without sequelae (AU)

A 12-year retrospective study of non-burn skin loss (burn-like syndromes) at a tertiary burns unit in a developing country.

BACKGROUND: A retrospective study of the presentation, etiology, and prognosis of non-burn epidermal loss managed at the Lagos University Teaching Hospital Nigeria over a 12-year period. MATERIALS AND METHODS: Admission records of patients managed for non-burn skin loss were retrieved from the medical records. Demographic details of the patients, the initial diagnosis, final diagnosis, treatment and outcome of treatment was noted. RESULTS: A total of 23 patients were identified, 17 (74%) had idiosyncratic drug reactions. Of this 17, 6 (26%) had Steven Johnson Syndrome, 6 (26%) had Steven Johnson Syndrome/toxic epidermal necrolysis while 5 (22%) presented with toxic epidermal necrolysis. Three of the five patients with toxic epidermal necrolysis died. The age range of patients with idiosyncratic adverse drug reactions was 2-28 years, mean, 10.18+/-1.44 years and male to female ratio of 1:1.83. The body surface area involved ranged from 8 to 78%; mean 26.65+/-6.08%. The agents suspected for the reactions were Co-trimoxazole (41.2%) and combination of Co-trimoxazole, and Fansidar (17.6%). Other conditions seen were two (9%) Staphylococcal Scalded Skin Syndrome, three (13%) had Necrotizing Faciitis, one of whom was HIV positive and died. One (4%) patient presented with pemphigus vulgaris. The presentation and management of the patients was discussed.

Staphylococcal scalded skin syndrome in an adult patient with T-lymphoblastic non-Hodgkin's lymphoma.

BACKGROUND: Staphylococcal scalded skin syndrome (SSSS) is an exfoliative dermatitis caused by Staphylococcus aureus infection. In contrast to infants, it is rarely observed in adults. SSSS in adults usually occurs in predisposed individuals such as those with renal failure or immunodeficiency, but has also been reported in otherwise healthy subjects. The reported mortality rate in adults is usually high because of serious underlying disease. PATIENT AND METHODS: We report a case of SSSS in a young female patient with T-lymphoblastic lymphoma, who survived this potentially lethal complication. CONCLUSIONS: To the best of our knowledge, this is the first case of SSSS in an adult patient with T-lymphoblastic non-Hodgkin's lymphoma. Clinicians should be aware of SSSS as a rare but potentially fatal disorder, particularly in adult patients with malignancies undergoing aggressive chemotherapy.

[Staphylococcal scalded skin syndrome and bullous impetigo in newborn twins infected by breast milk]. / Epidermolyse staphylococcique et impétigo bulleux chez des jumeaux nouveau-nés contaminés par le lait maternel.

Staphylococcus aureus is often responsible for late septic infections, more rarely of toxinic ones, occurring in neonatal period. We report a case of staphylococcal scalded skin syndrome and bullous impetigo in newborn twins infected by breast milk from their asymptomatic mother. This transmission was confirmed by molecular biology method. This case emphasizes the potential part of the mother in staphylococcal nosocomial infections and the complexity of toxinic mechanisms.

Mechanisms of blister formation by staphylococcal toxins.

Many children suffer from the bacterial skin diseases bullous impetigo and staphylococcal scalded skin syndrome (SSSS). Staphylococcus aureus, which produces exfoliative toxins (ETs), causes these diseases. Recently, it was proven that ETs cleave the cell adhesion molecule desmoglein (Dsg) 1, which plays an important role in maintaining the proper structure and barrier function of the epidermis. Surprisingly, Dsg1 is also the antibody target in the autoimmune disease pemphigus foliaceus. Skin biopsies from pemphigus foliaceus patients show the same pathology as those from bullous impetigo and SSSS patients. The crystal structure of ET suggests that it is a serine protease with an inactive catalytic site, which may become activated when ET binds a specific receptor. This receptor binding is thought to cause a change in conformation that exposes the catalytic site. It has recently been shown that Dsg1 specifically binds and activates ET, which in turn cleaves the bound Dsg1 at only one peptide bond. This process is absolutely dependent on the calcium-dependent conformation of Dsg1. These data suggest that ETs have a very high specificity for human Dsg1, and that S. aureus uses ETs to disrupt the barrier of the human epidermis in order to survive and proliferate on the human body.

Molecular mechanisms of blister formation in bullous impetigo and staphylococcal scalded skin syndrome.

Bullous impetigo due to Staphylococcus aureus is one of the most common bacterial infections of man, and its generalized form, staphylococcal scalded skin syndrome (SSSS), is a frequent manifestation of staphylococcal epidemics in neonatal nurseries. Both diseases are mediated by exfoliative toxins (ETs), which show exquisite pathologic specificity in blistering only the superficial epidermis. We show that these toxins act as serine proteases with extremely focused molecular specificity to cleave mouse and human desmoglein 1 (Dsg1) once after glutamic acid residue 381 between extracellular domains 3 and 4. Mutation of the predicted catalytically active serine to alanine completely inhibits cleavage. The mutated ETs bind specifically to Dsg1 by immunofluorescence colocalization and by coimmunoprecipitation. Thus, ETs, through specific recognition and proteolytic cleavage of one structurally critical peptide bond in an adhesion molecule, cause its dysfunction and allow S. aureus to spread under the stratum corneum, the main barrier of the skin, explaining how, although they circulate through the entire body in SSSS, they cause pathology only in the superficial epidermis.

Staphylococcal scalded skin syndrome in an adult associated with methicillin-resistant Staphylococcus aureus.

We report the first adult case of staphylococcal scalded skin syndrome (SSSS) due to methicillin-resistant Staphylococcus aureus (MRSA). This case is particularly unusual as the MRSA produced toxic shock syndrome toxin 1 and enterotoxin, but not exfoliatoxin. SSSS was originally described in neonates and is thought to result from exfoliatins which produce subcorneal splitting of the epidermis and are only produced by certain strains of S. aureus. This case reflects the range of toxins that can be associated with SSSS and the clinical manifestations of MRSA infection in adult patients.