Treatment decision-making for patients with the Herlitz subtype of junctional epidermolysis bullosa.

The Herlitz subtype of junctional epidermolysis bullosa (JEB-H) is a lethal genetic disorder characterized by recurrent and persistent erosions of the epithelial surfaces that heal with exuberant granulation tissue. In addition, respiratory distress, refractory anemia and failure to thrive are often seen. Mortality in the first year of life approaches 90%. JEB-H is caused by mutations in the genes that encode the protein laminin 5, a structural molecule involved in the adhesion of epidermis to dermis. There is currently no cure for JEB-H. Medical interventions treat complications but do not ultimately limit mortality. Ethical principles contend that offering comfort and company to the patient and family, not aggressive therapies, should comprise the mainstay of care for affected infants.

Induction of the skin endogenous protective mitochondrial MnSOD by Vitreoscilla filiformis extract.

Vitreoscilla filiformis (Vf), a filamentous bacteria living in fresh water is thought to contribute to the observed beneficial effects of Spa water on skin. An active fraction obtained from a Vf biomass was evaluated for its ability to modulate mRNA expression in cultured skin cells. cDNA array analysis was conducted first using a customized membrane including 1176 selected and fully identified genes involved in skin physiology and homeostasis then the newly developed full genome U133 plus 2.0 GeneChip from Affymetrix. The mitochondrial protective manganese superoxide dismutase (MnSOD/SOD-2) was identified as a preferentially induced mRNA target in both normal human dermal fibroblasts and keratinocytes. Induction at the transcriptional level in both cell types was confirmed using quantitative real time/polymerase chain reaction and a kinetic analysis revealed a maximal increase in mRNA expression 20 h after stimulation with Vf extract (Vfe). Using immunofluorescent (fluorescent cell sorter) analysis, an induction of MnSOD protein in both normal human dermal skin fibroblasts (x1.6; P < 0.01) and epidermal keratinocytes (x1.4; P < 0.01) was confirmed. As MnSOD is a major inducible free-radical scavenger in skin, these results suggest that the Vfe could induce skin cells to produce their own endogenous protective defences in vivo against both exogenous environmental stressors such as UV irradiation or microflora as well as to combat endogenous sources of deleterious free radicals involved in skin ageing. Finally, in order to confirm the in vivo potential of this original extract in human, we evaluated its protective activity vs. placebo on the generation of sunburn cells in epidermis under UVB stress. As expected from in vitro profiling, Vfe was indeed found to significantly inhibit the appearance of sunburn cells in UVB-exposed areas, a signature of skin alteration which has been suggested to be linked to a defect in MnSOD protective activity. Altogether, those data suggest that the combination of a suitable protective UV filter together with this bioactive Vfe might improve skin protection through complementary pathways.

Skin benefits from continuous topical administration of a zinc oxide/petrolatum formulation by a novel disposable diaper.

BACKGROUND: Diaper dermatitis is a common childhood affliction. Aiming to help reduce the prevalence of this problem, we have advanced in our development of a novel diaper that delivers dermatological formulations to help protect the skin from over-hydration and irritation. OBJECTIVE: To determine the clinical benefits of a novel disposable diaper designed to deliver a zinc oxide and petrolatum-based formulation continuously to the skin during use. METHODS: All studies were independent, blinded, randomized clinical trials. Study A was conducted to confirm transfer of the zinc oxide/petrolatum (ZnO/Pet) formulation from the diaper to the child's skin during use. Children wore a single diaper for 3 h or multiple diapers for 24 h. After the use period, stratum corneum samples were taken from each child and analysed for ZnO/Pet. Study B evaluated the prevention of skin irritation and barrier damage from a standard skin irritant (SLS) in an adult arm model. Study C evaluated skin erythema and diaper rash in 268 infants over a 4-week usage period. One half of the infants used the ZnO/Pet diaper, while the other half used a control diaper that was identical except for the absence of the ZnO/Pet formulation. RESULTS: The ointment formulation and ZnO transferred effectively from the diaper to the child's skin during product use. Transfer of ZnO increased from 4.2 microg/cm2 at 3 h to > 8 microg/cm2 at 24 h. Exposure to the formulations directly on adult skin prior to an irritant challenge was associated with up to a 3.5 reduction in skin barrier damage and skin erythema. Greatest reductions were seen for the ZnO containing formulations. Wearing of the formulation treated diaper was also associated with a significant reduction in skin erythema and diaper rash compared to the control product. CONCLUSIONS: The results demonstrated the clinical benefits associated with continuous topical administration of a zinc oxide/petrolatum-based formulation by this novel diaper.

Neonatal skin care: evaluation of the AWHONN/NANN research-based practice project on knowledge and skin care practices. Association of Women's Health, Obstetric and Neonatal Nurses/National Association of Neonatal Nurses.

OBJECTIVE: To develop and evaluate an evidence-based clinical practice guideline for assessment and routine care of neonatal skin, educate nurses about the scientific basis for practices recommended in the guideline, and design procedures that facilitate implementation of the project guideline into clinical practice. DESIGN: Descriptive report of the collaborative neonatal skin care research-based practice project of the Association of Women's Health, Obstetric and Neonatal Nurses and the National Association of Neonatal Nurses. SETTING: Neonatal intensive-care unit (NICU) and special-care nurseries and well-baby nurseries in 51 hospitals located throughout the United States. PARTICIPANTS: Member site coordinators (N = 51), nurses who work at the selected sites, and the neonates observed during both the pre- and postimplementation phases of the project (N = 2,820). METHOD: An evidence-based clinical practice guideline was developed, sites were selected from all respondents of the call for sites, site coordinator training was provided, data collection was facilitated by project-specific data collection tools, and the project was evaluated by the science team. MAIN OUTCOME MEASURES: Diversity and numbers of sites represented, patient representation, site coordinator knowledge of neonatal skin care pre- and postimplementation, use of project-designed implementation tools, satisfaction with project guideline and the data collection process, changes in practices and product use, and site coordinators' experiences during guideline implementation. RESULTS: Fifty-one sites completed the project, representing NICU, special-care, and well-baby nurseries in both academic and community hospital settings in 27 states. Registered nurses working in these sites totaled 4,754 full-time equivalent positions (FTEs) (in NICU/special-care and well-baby nurseries). Site coordinators demonstrated increased knowledge of research-based neonatal skin care and satisfaction with the implementation tools and data collection process. Product use changed, reflecting acquisition of new knowledge. Barriers to implementation of the guideline were identified. CONCLUSIONS: The AWHONN/NANN Neonatal Skin Care Research-Based Practice Project demonstrated increased knowledge among site coordinators who received training, facilitated changes in neonatal skin care as defined by the practice guideline, and thus advanced evidence-based clinical practice.

Neonatal skin care: clinical outcomes of the AWHONN/NANN evidence-based clinical practice guideline. Association of Women's Health, Obstetric and Neonatal Nurses and the National Association of Neonatal Nurses.

OBJECTIVE: To test the effectiveness of an evidence-based clinical practice guideline for neonatal skin care on selected clinical outcomes for newborns in neonatal intensive-care units (NICU), special-care units (SCU), and well-baby nurseries. DESIGN: Prospective evaluation of the collaborative neonatal skin care research-based practice project of the Association of Women's Health, Obstetric and Neonatal Nurses and the National Association of Neonatal Nurses. SETTING: NICU and well-baby units in 51 hospitals located throughout the United States. PARTICIPANTS: Member site coordinators (N = 51) and the neonates (N= 2,820) observed during both the pre- and postimplementation phases of the project. METHOD: Site coordinators received specialized education in neonatal skin care and implemented an evidence-based clinical practice guideline addressing 10 aspects of neonatal skin care. Baseline observations of skin condition, care practices, and environment of newly admitted neonates were collected by site coordinators. Postimplementation observations were then completed. MAIN OUTCOME MEASURES: Skin condition was assessed with the Neonatal Skin Condition Score (NSCS), which ranges from a score of three (best condition) to a score of nine (worst condition), based on dryness, erythema, and skin breakdown. Changes in frequency of selected skin care practices were used to assess the effectiveness and feasibility of using the practice guideline in everyday clinical practice. Aspects of the care environment with potential effect on skin integrity were monitored to determine risk factors. RESULTS: Fifty-one site coordinators made 11,468 systematic assessments of 2,464 NICU and SCU newborns and 356 well newborns. Baseline skin scores were better in well newborns compared with premature newborns. After implementation of the guideline, skin condition was improved, as reflected by less visible dryness, redness, and skin breakdown in both the NICU/SCU and well newborns. The guideline was integrated into care, as evidenced by increased use of emollients, particularly with premature infants, and decreased frequency of bathing. A relationship was shown between selected aspects of the environment and alterations in skin integrity. CONCLUSIONS: Use of the AWHONN/NANN Neonatal Skin Care Research-Based Clinical Practice Guideline was successfully implemented at 51 sites, and effectiveness was demonstrated by changed care practices and improved skin condition in premature and full-term newborns. The results of this project support a wider dissemination of the project's practice guideline for neonatal skin care.

Diaper dermatitis.

The primary goals of preventing and treating diaper dermatitis include keeping the skin dry, protected, and infection free. Frequent diaper changes with the superabsorbent disposable diapers may be the best tactic for infants' skin, if not the environment. Also, the more time that infants spend without diapers, the less dermatitis they experience, but a practical balance must be struck. Gentle cleansing and barrier creams are beneficial, and candidal infection must be treated. Finally, any recalcitrant diaper dermatitis must be further investigated to uncover underlying disease (Fig. 6).

Skin manifestations of mitochondrial DNA syndromes: case report and review.

Mitochondrial DNA syndromes are an emerging class of diseases that can present at any age. Clinical findings are legion and may include renal tubulopathy, growth retardation, myopathy, seizures, and ophthalmoplegia. Mitochondrial DNA syndromes have presented with symmetric cervical lipomas, poikiloderma, and anhidrosis. We describe a child with a novel mitochondrial DNA syndrome who had poikiloderma on sun-exposed areas. We also reviewed 274 patients with mitochondrial DNA disorders for skin findings. Symmetric cervical lipomas were consistently associated with myoclonic epilepsy as part of 1 syndrome. With the exception of lipomas, skin findings were reported in 16 patients.

A young boy with a large hemifacial plaque with histopathologic features of trichoepithelioma.

Trichoepitheliomas commonly appear as sporadic solitary lesions or, more rarely, as multiple lesions that are often dominantly inherited. We describe an 8-year-old boy with multiple facial papules that coalesced into a large plaque. This presentation of multiple trichoepitheliomas may represent an unusual type of nevus.

Warts and molluscum contagiosum: beware of treatments worse than the disease.

To treat or not to treat, that is the question. Two cutaneous infections, warts and molluscum contagiosum, have evaded eradication for centuries, and the viruses continue to thrive and to expand in number despite all attempts at destruction. Meanwhile, many cases regress spontaneously. In this article, the authors review the characteristics of the viruses involved; discuss their transmission, epidemiology, and clinical manifestations; and assess the effectiveness of available therapies.

Topical ointment therapy benefits premature infants.

OBJECTIVE: Premature infants have an ineffective epidermal barrier. The aim of this study was to investigate the cutaneous and systemic effects of preservative-free topical ointment therapy in premature infants. STUDY DESIGN: We conducted a prospective, randomized study of 60 infants less than 33 weeks' estimated gestational age. The treated infants received therapy for 2 weeks with twice-daily preservative-free topical ointment therapy while the control group received no topical treatment or as-needed therapy with a water-in-oil emollient. Data collection included transepidermal water loss (TEWL) measurement, skin condition evaluations, fungal and quantitative bacterial skin cultures, analysis of fluid requirements, patterns of weight low or gain, and the incidence of blood and cerebrospinal fluid cultures positive for microorganisms. RESULTS: We found that topical ointment therapy significantly decreased TEWL during the first 6 hours after the initial application. TEWL was decreased by 67% (p = 0.0001) when measured 30 minutes after application and 34% (p = 0.001) when measured 4 to 6 hours after application. We also observed significantly superior skin condition scores in the treated group on study days 7 and 14 (p = 0.001) and 0.0004, respectively). Quantitative bacterial cultures revealed significantly less colonization of the axilla on day 2, 3, or 4 and on day 14 (p = 0.008 and 0.04, respectively). The incidence of positive findings in blood and/or cerebrospinal fluid cultures was 3.3% in the treated group of infants versus 26.7% in the control group (p = 0.02). There was no statistical difference in the fluid requirements or patterns of weight gain or loss during the 2 weeks of the study. CONCLUSIONS: Preservative-free topical ointment therapy decreased TEWL for 6 hours after application, decreased the severity of dermatitis, and decreased bacterial colonization of axillary skin. Infants treated with ointment had fewer blood and cerebrospinal fluid cultures positive for microorganisms. These data support the use of topical ointment therapy in very premature infants during the first weeks after birth.

Impetigo: an overview.

This article reviews in detail the pathogenesis, clinical characteristics and management of impetigo in children. Impetigo is the most common bacterial skin infection of children. Most cases of nonbullous impetigo and all cases of bullous impetigo are caused by Staphylococcus aureus. The remainder of cases of nonbullous impetigo are due to group A beta hemolytic streptococci (GABHS). GABHS colonize the skin directly by binding to sites on fibronectin that are exposed by trauma. In contrast, S. aureus colonizes the nasal epithelium first; from this reservoir, colonization of the skin occurs. Patients with recurrent impetigo should be evaluated for carriage of S. aureus. Superficial, localized impetigo may be treated successfully in more than 90% of cases with topical application of mupirocin ointment. Impetigo that is widespread or involves deeper tissues should be treated with a beta-lactamase-resistant oral antibiotic. The choice of antibiotics is affected by the local prevalence of resistance to erythromycin among strains of S. aureus, antibiotic cost and availability, and issues of compliance.

Semipermeable dressings improve epidermal barrier function in premature infants.

Infants of less than 32 wk gestation have a defective epidermal barrier, with increased skin permeability and transepidermal water loss (TEWL). We studied the effect of a nonadhesive semipermeable dressing on the epidermal barrier of premature infants and on fetal skin transplanted to nude mice. Fifteen infants with a mean estimated gestational age of 27.7 wk and 16 human fetal skin grafts (estimated gestational age, 23-26 wk) transplanted to eight nude mice were studied. One lower leg (or skin graft) was treated and the other left untreated as a control. In the infants, TEWL was measured on control skin and treated skin (both through the dressing and after temporary dressing removal) on d 0, 1, 2, 4, and 7. Bacterial and fungal cultures were also performed. In the mice, TEWL and skin blood flow were measured on d 0, 2, and 4. Biopsies were obtained on d 4 for a cell proliferation assay, histology, and electron microscopy. Treated infant skin showed a consistently lower bacterial number and a significantly decreased TEWL (measured through the dressing). There was also a significantly lower TEWL on the treated side, measured after temporary dressing removal, on d 1, 2, 4, and 7, documenting improved epidermal barrier function. The animal study revealed decreased TEWL and a nearly 2-fold greater d-4 keratinocyte proliferation (p = 0.01) in treated skin and decreased blood flow on d 4 in control skin (p = 0.01). There was no significant difference in the volume density of membrane coating granules or the morphology of intercorneocyte spaces.(ABSTRACT TRUNCATED AT 250 WORDS)

Proteus syndrome.

A 10-month-old girl had macrodactyly, facial and extremity hemihypertrophy, plantar cerebriform hyperplasia, a subcutaneous mass on the back, macrocephaly, and lumbar kyphosis. These findings were diagnostic of Proteus syndrome. The clinical features, etiology, management, and points of differential diagnosis are discussed.

Effects of repeated application of emollient cream to premature neonates' skin.

OBJECTIVE: Emollient cream moisturizers are often used on premature newborns in neonatal intensive care units without accurate knowledge of the risks or benefits to the neonate. DESIGN: We prospectively compared premature neonates treated with a water-in-oil emollient cream for up to 16 days to untreated premature neonates. SETTING: The study was completed in a neonatal intensive care unit on neonates admitted for respiratory distress and/or possible sepsis. PATIENTS: Thirty-four neonates, between 29 and 36 weeks estimated gestational age, entered the study. INTERVENTIONS: One-half of the neonates were treated twice a day with an water-in-oil emollient cream, and the other half served as controls. OUTCOME MEASURES: The skin condition of the neonates' hands, feet, and abdomen was evaluated on entering the study and twice a week. Fungal cultures and quantitative bacterial cultures were obtained from the axilla and abdomen on entering the study and twice a week. RESULTS: The mean gestational age of the treated neonates was 32.3 weeks, whereas the mean gestational age of the control neonates was 32.5 weeks. The neonates treated with emollient cream demonstrated statistically less dermatitis of their hands (day 2 through day 11), their feet (day 2 through day 16), and their abdomen (day 7 through day 11). Fungal cultures and quantitative bacterial cultures of the abdomen and axilla were equivalent in both groups. CONCLUSIONS: These studies document that emollient cream moisturizer therapy of premature neonates decreases dermatitis without changing the microbiological flora.

Candida and Malassezia as nursery pathogens.

Candida and Malassezia can be both normal flora and pathogens in the nursery. Very low-birth-weight infants are at high risk for systemic infection from these organisms. We review the microbiology and clinical manifestations of disease within the neonatal nursery.