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Erythroderma in children: a clinico-etiological study.

Although there are various published studies on erythroderma from western and Asian countries, most of them have only included patients in the adult age groups. As we have an exclusively pediatric dermatology unit, we thought it would be intriguing to study the clinical, etiological and laboratory parameters of erythroderma in children. Seventeen erythroderma patients of both sexes were inducted into the study between 1993 to 1998. The mean age of onset was 3.3 years and the male:female ratio was 0.89:1. Eight (47%) of the patients were infants; 9 (53%) others belonged to the preschool and school going age group (age range between 1 to 12 years). An acute onset of the disease was seen in 47% of the patients while 53% of the patients had a chronic onset. The main presenting complaints were itching in 41% and burning in 18% of patients. Scalp involvement (71%), nail involvement (18%), and alopecia (6%) were the main cutaneous features observed while fever (53%), tachycardia (53%), pedal edema (12%), lymphadenopathy (18%), and hepatomegaly (12%) were the main systemic features observed in this study. Etiologically, drugs (29%), showed the highest incidence, followed equally (18%) by genodermatoses, psoriasis, and staphylococcal scalded skin syndrome (SSSS). Two (12%) patients had erythroderma due to atopic dermatitis, while one was (5%) due to infantile seborrheic dermatitis coexisting with dermatophytosis. Laboratory parameters contributed little towards diagnosis of the underlying dermatological condition. Thus, though erythroderma is a striking entity, it is yet uncommon in the pediatric age group. Because the drug induced group was the largest in this study, we recommend that drugs should be suspected as important causative factors of erythroderma in children.

Age Distribution↗

A simple and rapid method for purifying staphylococcal exfoliative toxin A.

A rapid and efficient method of purification of staphylococcal exfoliative toxin A, the causative agent of staphylococcal scalded skin syndrome (SSSS), has been developed. It is based on ammonium sulfate precipitation of the culture supernatant and hydrophobic interaction chromatography on Phenyl-Sepharose CL-4B. This procedure results in 87-fold purification of this toxin, which appears as a single band in sodium dodecyl sulfate-polyacrylamide gels.

Ammonium Sulfate↗

The fate of Staphylococcal exfoliatin in newborn and adult mice.

The distribution and excretion of the staphylococcal exfoliatin was investigated following in vivo administration of highly purified 125I-labelled exfoliatin fractions to adult and newborn mice. Adult mice excrete approximately one-third of a test dose by 3 hours as compared to a fifteenth of a test dose excreted by newborn mice. Accordingly, blood tracer radioactivity reaches a relatively higher peak and shows a slower decline in newborns than in adults. The urine of adult mice contains considerable biologically active exfoliating material. Both nephrectomized and carbon tetrachloride-poisoned adult mice injected with exfoliatin develop generalized exfoliation whereas comparable doses in untreated controls have no effect. On the other hand, subtotal hepatectomy, followed by injection of exfoliatin, does not lead to exfoliation. We conclude that renal immaturity is a critical factor responsible for the susceptibility of neonates to generalized staphylococcal scalded skin syndrome.

Age Factors↗

The experimental production of high-level intraepidermal splits.

The effects of trypsin at 37 degrees C and 4 degrees C on adhesion between keratinocytes in normal human epidermis were assessed by light and electron microscopy. The results provide evidence for the contribution of both extracellular and intracellular factors to keratinocyte adhesion and for changes in the nature of adhesion in the granular layer. Trypsinization at 4 degrees C produced a high-level intraepidermal split identical in location and intercellular route to that observed in the staphylococcal scalded skin syndrome.

Cell Adhesion↗

The differentiation and proliferation of newly formed epidermis on wounds treated with cultured epithelial allografts.

Fifteen patients, eight with burn or scald wounds and seven with split-thickness donor sites, were treated with cultured epithelial allografts. Skin was obtained from HIV-negative donors undergoing circumcision and sheets of epithelium were cultured using the 3T3 feeder method. Multiple post-operative biopsies were performed at various time intervals and stained with a panel of monoclonal antibodies against cytokeratins, involucrin, transferrin receptor and epidermal growth factor receptor. Fresh cultured epithelial sheets, normal skin, standard treated donor sites and burns treated with autografts were also studied. Cytokeratin-10 expression was not observed at treated sites until 4 weeks post-grafting, when normal suprabasal levels were observed. Cytokeratins 13 and 16, usually observed in highly proliferative states such as psoriasis, were observed in epithelial-treated sites for up to 6 months. Other proliferation markers such as Ki67 and transferrin receptor were only expressed 2-3 weeks post-operatively. Involucrin, a marker of keratinocyte terminal differentiation, was expressed throughout newly formed epidermis until 15 weeks, when the normal pattern of granular expression was observed. These results indicate that although the cultured 'allograft' does not survive, it may modulate the proliferation and differentiation of spontaneously regenerating epithelium.

Adult↗

Cloning of swine desmoglein 1 and its direct proteolysis by Staphylococcus hyicus exfoliative toxins isolated from pigs with exudative epidermitis.

Exudative epidermitis (EE) is an acute, often fatal skin disease of piglets caused by Staphylococcus hyicus. Clinical and histopathological manifestations of EE are similar to those of staphylococcal scalded skin syndrome (SSSS), a human blistering skin disease, in which exfoliative toxins produced by Staphylococcus aureus digest the extracellular domains of desmoglein (Dsg) 1 and cause loss of epidermal cell-cell adhesion. The aims of this study were to isolate and characterize cDNA for full length of swine Dsg1, and to determine whether the extracellular domains of swine Dsg1 produced by baculovirus (sDsg1-His) could be digested by four isoforms of exfoliative toxin produced by S. hyicus (ExhA, ExhB, ExhC and ExhD). Nucleotide sequencing revealed that swine Dsg1 cDNA consisted of an open reading frame of 3138 bp, encoding a precursor protein of 1045 amino acids. Deduced amino acid sequence of the swine Dsg1 precursor were highly homologous to corresponding bovine, canine, human and murine sequences. Immunoadsorption assay with a secreted form of sDsg1-His revealed that sDsg1-His specifically absorbs the immunoreactivity of 10 human pemphigus foliaceus sera against swine keratinocyte cell surfaces, suggesting its proper conformation. When sDsg1-His was incubated in vitro with Exhs, all four isoforms of Exh directly digested sDsg1-His into smaller peptides, whereas removal of calcium from sDsg1-His completely inhibited its proteolysis by these four Exhs. Recognition and digestion of calcium-stabilized structure on the extracellular domains of swine Dsg1 by Exhs indicated that EE shares similar molecular pathophysiological mechanisms of intra-epidermal splitting with SSSS in humans.

Amino Acid Sequence↗

Band-like opacity in the corneas of abattoir-acquired pig eyes.

In the course of an investigation into the distribution of immune cells in the porcine cornea, a band-like lesion on the cornea of two-thirds of the eyes acquired from the local abattoir was noted. Histological investigations revealed an area of corneal epithelium debridement with no obvious other pathological changes. Discussions with abattoir staff soon revealed the cause of the lesions to be the scalding process that all pig carcasses undergo immediately post-mortem that serves to remove unwanted hair and reduce skin-dwelling bacterial contamination. We concluded that the band-like opacity was the result of thermal injury to the anterior surface of the cornea between the open eyelids. It is hoped that this short communication will act as a cautionary note to other investigators who currently use or are considering using porcine eyes in research or as a model in surgical training and who may be unaware of the conventional handling procedures in pig abattoirs.

Abattoirs↗

The epidemiology of home injuries to children under five years in New Zealand.

OBJECTIVE: This paper describes the epidemiology of injuries sustained by children under five in the home. METHODS: Cases were selected from the New Zealand Health Information Service public hospital morbidity and mortality data, and included all 0-4 year olds where the place of injury occurrence was classified as 'home'. The circumstances of injury were coded according to the Supplementary Classifications of External Causes of Injury and Poisoning (E-codes) of the International Classifications of Diseases. Age-specific rates of death or hospitalisation due to injury were calculated using the population of 0-4 year olds in New Zealand for each year as the denominator. RESULTS: The rate of death from an injury sustained at home between 1989 and 1998 was 13 per 100,000 population per year. The main causes of death were suffocation, submersion, homicide and fire. The rate of hospitalisation in children aged 0-4 years from an injury sustained in the home between 1989 and 2000 was 737 per 100,000 population per year. The most frequently recorded causes of hospitalisation were falls, scalds, poisonings and cut/piercing incidents. CONCLUSION AND IMPLICATIONS: Although there has been an apparent decrease in the number of children hospitalised for injuries sustained in the home environment, it is not possible to determine if this is a 'real' change or a result of other factors affecting the data. While children continue to be killed and injured as a result of preventable incidents in the home environment, injury prevention strategies should be continued and strengthened.

Accident Prevention↗

Spina bifida: implications for 100 children at school.

One hundred children with spina bifida were examined at home and reviewed at the schools they attended. The severity of the handicap had not been appreciated at the time of school entry. 27 of the 41 children who were over 10 years of age had become wheel-chair dependent; 39 of the 100 children had an IQ below 80, and incontinence remained a problem for 68 children. 64 had visual defects, including two who were totally blind, 27 had epilepsy, and 87 had suffered fractures, burns, scalds or pressure sores. Most of the children had started at an ordinary school. The teachers had to give a disproportionate amount of attention to the handicapped child, but were themselves often given inadequate information and support. Many children had learning difficulties. As they grew older their incontinence was less well tolerated. Wheelchair dependency precluded their admission to an ordinary secondary school with stairs, so the majority of older children attended special schools. Only very few will enter normal employment, and many will require a period of education and training beyond the usual school-leaving age. The education of such children should have realistic goals and aim at achieving the maximum degree of self-care.

Activities of Daily Living↗

Random-amplified polymorphic DNA (RAPD) analysis shows intraspecies differences among Xanthomonas albilineans strains.

Five strains of Xanthomonas albilineans, causal agent of leaf scald disease in sugarcane from various geographical regions, were compared using random amplification of polymorphic DNA (RAPD) to determine whether they could be differentiated at the DNA level. CsCl-purified genomic DNA from these strains were amplified by the polymerase chain reaction (PCR) using arbitrary 10-mer primers according to standard RAPD conditions and the amplification product profiles analysed by conventional agarose gel electrophoresis. Although most RAPD markers were common to all five strains, unique profiles for each strain were discernible using four 10-mer arbitrary primers individually. Reproducible DNA fingerprints indicate that RAPD analysis can be used to identify and differentiate the X. albilineans strains. This technique has the potential for use in monitoring the appearance of foreign strains of X. albilineans in various geographical regions and could be used for the construction of phylogenetic trees.

DNA Fingerprinting↗

Thermal trauma alters myocardial cyclic nucleotides and protein content in mice.

1. This study tested the hypothesis that the systemic effects of burn include altered metabolic activity in the heart. Metabolic activity was studied by measuring alterations in cyclic nucleotide levels and protein concentrations in atrial and ventricular muscle in mice at 14 and 22 days after a 20% body surface area (BSA) burn. Thermal injury was produced on the dorsal surface of anesthetized male CD mice by immersion in water at 95 degrees C for 8 s. This resulted in a full-thickness, 3 degrees scald burn. In atrial and ventricular tissues, levels of adenosine 3':5'-cyclic monophosphate (cyclic AMP) and guanosine 3':5'-cyclic monophosphate (cyclic GMP) were analyzed by 125I-radioimmunoassay. 2. The protein content (mg prot g-1 dry wt) increased in the atria. The cyclic AMP content (nmol g-1 dry wt) was significantly increased fourfold and ninefold at 14 and 22 days, respectively, in atria from burned animals compared to controls. The cyclic AMP/cyclic GMP ratios were similarly increased. 3. In the ventricle, the protein content and cyclic AMP levels were not altered, but the cyclic AMP/cyclic GMP ratios (nmol g-1 dry wt) were increased at both 14 and 22 days. These changes both in atria and ventricles were less prominent when cyclic nucleotide concentrations or ratios were expressed as pmol mg-1 protein. 4. The data confirm the hypothesis that a 20% BSA thermal injury evokes effects in sites remote from burn injury such as in the atria and ventricles. These effects include total body weight loss, elevated cyclic AMP, cyclic AMP/cyclic GMP ratios, and protein levels in the atria, and elevated cyclic AMP/cyclic GMP ratios in both atrial and ventricular tissues at 2 and 3 weeks after thermal injury. To prevent underestimation of cyclic nucleotide levels such changes should preferably be expressed on a prot g- dry weight basis.

Animals↗

Toxic epidermal necrolysis due to antiepileptic therapy in an 8-year-old boy.

Toxic epidermal necrolysis (TEN) has rarely been documented in children. We treated an 8-year-old boy who developed generalized blistering dermatosis after anticonvulsive therapy with diphenylhydantoin. The diagnosis was proved by exfoliative cytology, histology, and electron microscopy. The disease entity and its differentiation from staphylococcal scalded-skin syndrome and toxic shock syndrome are reviewed.

Adult↗

Toxic epidermal necrolysis in a 6-week-old infant.

Toxic epidermal necrolysis was documented in a 6-week-old infant with Klebsiella pneumoniae sepsis who received many medications. We inoculated infant mice with the K. pneumoniae isolate but were unable to produce histologic changes resembling those seen in our patient. This condition should be included in the differential diagnosis of severe drug reactions in very young infants with clinical scalded-skin syndromes.

Animals↗

Generalized bullous impetigo in a neonate.

Generalized bullous impetigo is uncommon in healthy and term neonates. It must be differentiated from staphylococcal scalded skin syndrome and other commonly encountered vesiculopustular lesions. We report generalized bullous impetigo in an otherwise healthy neonate.

Anti-Bacterial Agents↗

Risk factors for burns as a consequence of seizures in persons with epilepsy.

To explore the risk factors and circumstances for burns in a population of persons with epilepsy, we surveyed 244 University of Colorado Health Sciences Center Seizure Clinic patients. Twenty-five had at least one seizure-related burn requiring medical attention (12 were hospitalized), 21 reported burns requiring medical attention not related to seizure activity, and 199 reported no burns. All patients with seizure-related burns had alteration of consciousness during most seizures. Statistical models with logistic regression indicated that a useful model for assessing risk of seizure-related burns in this population of patients could be constructed using three significant variables; lifetime total number of seizures experienced by the patient, the presence of interictal neurologic impairment, and gender. The remainder of the variables studied (percentage of seizures that occurred nocturnally, percentage of seizures preceded by a simple partial seizure (SPS) warning, self-care status, number of seizures in adult life, seizure type, patient age, age of onset of seizure disorder, and presence of burns not associated with seizure) were either not significantly correlated with seizure-related burns or did not significantly improve the success of the statistical model in light of the other variables studied. Most injuries occurred in the home during activities that might have been avoided. They included 10 burns incurred while cooking, 5 incurred while showering (with a plumbing system that permitted exposure to scalding water), and 3 caused by exposed room heaters. A subset of epileptic patients are at increased risk for burns and should minimize situations in which they can be burned.

Accident Prevention↗

Patients' experiences of injury as a result of epilepsy.

PURPOSE: The increased risk of mortality among people with epilepsy is well documented; people with epilepsy are more likely than the general population to die as a result of an accident. Data about incidence of nonfatal accidents and associated factors are not so readily available, even though such accidents are more common than fatal injuries. We report the proportion of people who sustain various injuries during a seizure and the key variables predicting injury. METHODS: Questionnaires were mailed to an unselected, community-based population of patients with epilepsy. The questionnaire included clinical and demographic details, previously validated scales of psychosocial well-being, and questions about seizure-related injuries. RESULTS: Of patients who had had at least one seizure during the previous year, 24% sustained at least one head injury, 16% sustained a burn or scald, 10% a dental injury, and 6% some other fracture. Seizure type, seizure severity, and seizure frequency were key predictors of having sustained at least one of these four seizure-related injuries. Key predictors of burn/scald were seizure severity, seizure frequency and sex; those of head injury were seizure severity and type; that of dental injury was seizure severity; and those of some other fracture were seizure severity, duration of epilepsy, and three or more drug-related adverse effects. CONCLUSIONS: These data help identify significant risk factors associated with seizure-related injuries and so facilitate sensible patient counseling about how the risks of such injuries can be minimized.

Accidents↗

Neonatal skin barrier: structure, function, and disorders.

The development of the human skin from intrauterine to extrauterine life is a balletic interplay of maturing layers and interlocking structures. We discuss this transition and then branch out to touch on issues of premature infant as well as neonatal skin care. Disruption of the barrier function due to toxins and development errors are expounded upon. Staph scalded skin syndrome, collodion membrane, bullous congenital ichthyosiform erythroderma, autosomal recessive ichthyosis (lamellar and congenital ichthyosiform erythroderma), and harlequin fetus are used as examples of these disruptions. Discussion of therapy with the authors' experience highlights each disease.

Humans↗

Urinary bladder marsupialization for treatment of obstructive urolithiasis in male goats.

OBJECTIVES: To describe a surgical procedure for urinary bladder marsupialization and to report the results obtained from its use in the treatment of obstructive urolithiasis in male goats. STUDY DESIGN: Retrospective evaluation. ANIMALS OR SAMPLE POPULATION: Male goats with obstructive urolithiasis. METHODS: Medical records of male goats that had urinary bladder marsupialization for the treatment of obstructive urolithiasis were reviewed. Data retrieved from the medical records included signalment, postoperative treatment, duration of hospitalization, and short-term and long-term complications. Median values for measured variables were calculated. RESULTS: A total of 18 of 19 goats survived. Urinary flow was re-established in all 19 goats at the conclusion of surgery. Short-term postoperative complications (bladder mucosal prolapse and death) were observed in 2 goats. Long-term postoperative complications (cystitis and fibrotic stomal closure) occurred in 2 animals. Median duration of hospitalization was 4 days. At the time of follow-up, mild urine scald was reported for all goats. Clinical signs of upper urinary tract disease or obstruction were not reported. A total of 15 of 17 owners were satisfied with the procedure. CONCLUSIONS: Urinary bladder marsupialization provided long-term resolution of urinary outflow obstruction in all goats with acceptable morbidity. CLINICAL RELEVANCE: Urinary bladder marsupialization is a procedure that provides a good prognosis for long-term resolution of obstructive urolithiasis in male goats.

Animals↗