Prevalence, pathophysiology and management of itch in epidermolysis bullosa.

Epidermolysis bullosa (EB) is a highly diverse group of inherited skin disorders, resulting from mutations in genes encoding proteins of the dermoepidermal junction. Itch (pruritus) is one of the most common symptoms across all EB subtypes. It occurs in blistered or wounded sites, or manifests as a generalized phenomenon, thereby affecting both intact skin and healing wounds. The mechanism of pruritus in EB is unclear. It is likely that skin inflammation secondary to barrier disruption, wound healing cascades and dysregulated activation of epidermal sensory nerve endings are all involved in its pathophysiology on the molecular and cellular level. Understanding these mechanisms in depth is crucial in developing optimized treatments for people with EB and improving quality of life. This review summarizes current evidence on the prevalence, mechanisms and management of itch in EB.

Recommendations for rosacea diagnosis, classification and management: update from the global ROSacea COnsensus 2019 panel.

BACKGROUND: A transition from a subtyping to a phenotyping approach in rosacea is underway, allowing individual patient management according to presenting features instead of categorization by predefined subtypes. The ROSacea COnsensus (ROSCO) 2017 recommendations further support this transition and align with guidance from other working groups. OBJECTIVES: To update and extend previous global ROSCO recommendations in line with the latest research and continue supporting uptake of the phenotype approach in rosacea through clinical tool development. METHODS: Nineteen dermatologists and two ophthalmologists used a modified Delphi approach to reach consensus on statements pertaining to critical aspects of rosacea diagnosis, classification and management. Voting was electronic and blinded. RESULTS: Delphi statements on which the panel achieved consensus of ≥ 75% voting 'Agree' or 'Strongly agree' are presented. The panel recommends discussing disease burden with patients during consultations, using four questions to assist conversations. The primary treatment objective should be achievement of complete clearance, owing to previously established clinical benefits for patients. Cutaneous and ocular features are defined. Treatments have been reassessed in line with recent evidence and the prior treatment algorithm updated. Combination therapy is recommended to benefit patients with multiple features. Ongoing monitoring and dialogue should take place between physician and patients, covering defined factors to maximize outcomes. A prototype clinical tool (Rosacea Tracker) and patient case studies have been developed from consensus statements. CONCLUSIONS: The current survey updates previous recommendations as a basis for local guideline development and provides clinical tools to facilitate a phenotype approach in practice and improve rosacea patient management. What's already known about this topic? A transition to a phenotype approach in rosacea is underway and is being recommended by multiple working groups. New research has become available since the previous ROSCO consensus, necessitating an update and extension of recommendations. What does this study add? We offer updated global recommendations for clinical practice that account for recent research, to continue supporting the transition to a phenotype approach in rosacea. We present prototype clinical tools to facilitate use of the phenotype approach in practice and improve management of patients with rosacea.

Updating the diagnosis, classification and assessment of rosacea: recommendations from the global ROSacea COnsensus (ROSCO) panel.

BACKGROUND: Rosacea is currently diagnosed by consensus-defined primary and secondary features and managed by subtype. However, individual features (phenotypes) can span multiple subtypes, which has implications for clinical practice and research. Adopting a phenotype-led approach may facilitate patient-centred management. OBJECTIVES: To advance clinical practice by obtaining international consensus to establish a phenotype-led rosacea diagnosis and classification scheme with global representation. METHODS: Seventeen dermatologists and three ophthalmologists used a modified Delphi approach to reach consensus on statements pertaining to critical aspects of rosacea diagnosis, classification and severity evaluation. All voting was electronic and blinded. RESULTS: Consensus was achieved for transitioning to a phenotype-based approach to rosacea diagnosis and classification. The following two features were independently considered diagnostic for rosacea: (i) persistent, centrofacial erythema associated with periodic intensification; and (ii) phymatous changes. Flushing, telangiectasia, inflammatory lesions and ocular manifestations were not considered to be individually diagnostic. The panel reached agreement on dimensions for phenotype severity measures and established the importance of assessing the patient burden of rosacea. CONCLUSIONS: The panel recommended an approach for diagnosis and classification of rosacea based on disease phenotype.

Racial disparities in cord blood vitamin D levels and its association with small-for-gestational-age infants.

OBJECTIVE: To examine the relationship of race and maternal characteristics and their association with cord blood vitamin D levels and small-for-gestational-age (SGA) status. STUDY DESIGN: Cord blood vitamin D levels were measured in 438 infants (276 black and 162 white). Multivariable logistic regression models were used to evaluate associations between maternal characteristics, vitamin D status and SGA. RESULTS: Black race, Medicaid status, mean body mass index at delivery and lack of prenatal vitamin use were associated with vitamin D deficiency. Black infants had 3.6 greater adjusted odds (95% confidence interval (CI): 2.4, 5.6) of vitamin D deficiency when compared with white infants. Black infants with vitamin D deficiency had 2.4 greater adjusted odds (95% CI: 1.0, 5.8) of SGA. Vitamin D deficiency was not significantly associated with SGA in white infants. CONCLUSION: Identification of risk factors (black race, Medicaid status, obesity and lack of prenatal vitamin use) can lead to opportunities for targeted prenatal vitamin supplementation to reduce the risk of neonatal vitamin D deficiency and SGA status.

Case mix measures and diagnosis-related groups: opportunities and threats for inpatient dermatology.

OBJECTIVE: The changing healthcare environment world-wide is leading to extensive use of per case payment systems based on diagnosis-related groups (DRG). The aim of this study was to examine the impact of application of different DRG systems used in the German healthcare system. METHODS: We retrospectively analysed 2334 clinical data sets of inpatients discharged from an academic dermatological inpatient unit in 2003. Data were regarded as providing high coding quality in compliance with the diagnosis and procedure classifications as well as coding standards. The application of the Australian AR-DRG version 4.1, the German G-DRG version 1.0, and the German G-DRG version 2004 was considered in detail. To evaluate more specific aspects, data were broken down into 11 groups based on the principle diagnosis. MAIN OUTCOME MEASURE: DRG cost weights and case mix index were used to compare coverage of inpatient dermatological services. Economic impacts were illustrated by case mix volumes and calculation of DRG payments. RESULTS: Case mix index results and the pending prospective revenues vary tremendously from the application of one or another of the DRG systems. The G-DRG version 2004 provides increased levels of case mix index that encourages, in particular, medical dermatology. CONCLUSIONS: The AR-DRG version 4.1 and the first German DRG version 1.0 appear to be less suitable to adequately cover inpatient dermatology. The G-DRG version 2004 has been greatly improved, probably due to proceeding calculation standards and DRG adjustments. The future of inpatient dermatology is subject to appropriate depiction of well-established treatment standards.

Comparison of different culture media and storage temperatures for the long-term preservation of Streptococcus pneumoniae in the tropics.

OBJECTIVE: The preservation of Streptococcus pneumoniae by standard freezing methods for subsequent tests--such as serotyping and antibiotic susceptibility--is not possible or is difficult in many developing countries because of the high cost of equipment, inadequate equipment maintenance, and irregular power supply. We evaluated alternative low-cost methods, by comparing different culture media and storage temperatures. METHODS: Clinical isolates of five capsular types (1, 5, 7, 19, and 23) of S. pneumoniae were preserved in rabbit blood, sheep blood, skimmed milk, or glycerol-chocolate broth, and stored at -20 degrees C or -70 degrees C. The cultures were also preserved by lyophilization or sand desiccation, followed by storage at room temperature and 4 degrees C. The viability of the preserved cultures was determined by making serial colony counts on day 0 and after 1 week, 4 weeks, 4 months and 16 months. The viability of cultures preserved by sand desiccation and storage at 4 degrees C was also determined every 6 months for up to 68 months. FINDINGS: Irrespective of the media used, cultures maintained at -20 degrees C became nonviable by the fourth month, while those maintained at -70 degrees C were still viable at 16 months. Cultures preserved by lyophilization or sand desiccation lost their viability by the fourth month when maintained at local room temperature (30-42 degrees C), but remained viable when stored at 4 degrees C for up to 68 months. CONCLUSIONS: Our results confirm that freezing at -70 degrees C, or lyophilization and storage at 4 degrees C are the ideal methods for the preservation of S. pneumoniae. In laboratories where lyophilization is not feasible, sand desiccation and storage at 4 degrees C offers an alternative low-cost method for the long-term preservation of S. pneumoniae.

Age- and cause-specific childhood mortality in Lombok, Indonesia, as a factor for determining the appropriateness of introducing Haemophilus influenzae type b and pneumococcal vaccines.

Using age and cause-specific childhood mortality in Lombok, Indonesia, as a factor for determining the appropriateness of introducing Haemophilus influenzae type b (Hib) and pneumococcal vaccines, the study describes a cross-sectional, hamlet-level mortality survey in 40 of 305 villages in Lombok Island, Indonesia. Causes of death were assessed with a standardized verbal-autopsy questionnaire. One thousand four hundred ninety-nine births and 141 deaths occurring among children aged less than 2 years were identified, with 43% of deaths occurring during the first 2 months of life. The infant mortality rate was 89 (95% CI: 75, 104) per 1,000 live-births. All mortality rates are reported per 1,000 live-births. To examine children whose deaths could potentially have been prevented through vaccination with Hib or pneumococcal vaccine, deaths due to acute respiratory infection (ARI) and central nervous system (CNS) infections among children, aged 2-23 months, were analyzed. ARI and CNS infections caused 58% (mortality rate: 31 per 1,000 live-births; 95% CI: 23, 41) and 17% (mortality rate: 9 per 1,000 live-births; 95% CI: 5, 16), respectively, of all deaths within this age group. Between the ages of 2 and 23 months, 5% of all babies born alive died of ARI, and another 1% died of CNS infections. Our results indicate that current efforts to reduce childhood mortality should focus on reducing ARI and meningitis. These efforts should include evaluating the impact of Hib and pneumococcal vaccines within the routine Expanded Programme on Immunization system.

Serotyping of Streptococcus pneumoniae by agglutination assays: a cost-effective technique for developing countries.

There is a need for additional data on the distribution of pneumococcal serotypes in developing countries. We report the use of a coagglutination (COA) and a latex agglutination (LA) test for serotyping Streptococcus pneumoniae which were evaluated using 114 clinical isolates in Vellore, India. In tests to serotype 30 fresh isolates of pneumococci from meningitis (8 isolates), bacteraemia/septicaemia (21 isolates) and peritonitis (1 isolate) cases, there was complete concordance among the three methods. An additional 20 isolates (11 from cerebrospinal fluid and 9 from blood cultures) were serotyped using both LA and COA, with full agreement between the results. With a further 30 isolates, there was 93% concordance for the COA types with serotypes assigned by a WHO reference laboratory. The COA and LA serotyping results were equivalent in accuracy to those obtained using quellung serotyping. Both these agglutination tests are rapid, valid, and relatively cheap, and with appropriate validation by reference laboratories they could be more widely used in developing countries to obtain local and regional data on pneumococcal serotype distribution.