J.-F. Stalder

Clinical Validation and Guidelines for the SCORAD Index: Consensus Report of the European Task Force on Atopic Dermatitis

BACKGROUND We have previously reported how the SCORAD index was designed. This cumulative index combines objective (extent and intensity of lesions) and subjective (daytime pruritus and sleep loss) criteria. AIMS To study interobserver variability in scoring for objective SCORAD criteria and to optimize the scoring guidelines. MATERIAL AND METHODS Three scoring sessions were organized in 1993-1994 in Hamburg, Bordeaux and Rotterdam totalizing 19 patients (14 children and 5 adults) and 23 physicians, among whom 12 participated in at least 2 scoring sessions; 169 evaluation sheets have been processed using the SCORAD File Marker Pro software. At each session, total body photographs and close-up views were taken of each patient, and this material was reviewed at the final evaluation. RESULTS The extent of lesions according to the rule of nines showed interobserver variability mostly for patients with lesions of moderate intensity involving 20-60% of body surface. Intensity items were scored with more consistency overall, but variations subsided especially for oozing/crusts and lichenifications. Low and high scorer profiles and the benefit of training were noted. CONCLUSIONS This study has allowed to optimize clinical scoring using the SCORAD system. A proposal has been made to grade the severity of atopic dermatitis according to objective criteria in three groups for inclusion in clinical trials. The SCORAD index remains the major criterion for follow-up in trials.

Pediatric Mastocytosis Is a Clonal Disease Associated with D816V and Other Activating c-KIT Mutations

Adult mastocytosis is an incurable clonal disease associated with c-KIT mutations, mostly in exon 17 (D816V). In contrast, pediatric mastocytosis often spontaneously regresses and is considered a reactive disease. Previous studies on childhood mastocytosis assessed only a few patients and focused primarily on codon 816 mutations, with various results. In this study, we analyzed the entire c-KIT sequence from cutaneous biopsies of 50 children with mastocytosis (ages 0-16 years). A mutation of codon 816 (exon 17) was found in 42% of cases, and mutations outside exon 17 were observed in 44%. Unexpectedly, half of the mutations were located in the fifth Ig loop of c-KITs extracellular domain, which is encoded by exons 8 and 9. All mutations identified in this study were somatic and caused a constitutive activation of c-KIT. There was no clear phenotype-genotype correlation, no clear relationship between the mutations and familial versus spontaneous disease, and no significant change in the relative expression of the c-KIT GNNK+ and GNNK isoforms. These findings strongly support the idea that, although pediatric mastocytosis can spontaneously regress, it is a clonal disease most commonly associated with activating mutations in c-KIT.

Topical corticosteroid phobia in atopic dermatitis: a study of its nature, origins and frequency

Background  Topical corticosteroids remain the mainstay of atopic dermatitis therapy. Many atopic dermatitis therapeutic failures appear to be attributable to poor adherence to treatment due to topical corticosteroid phobia.

Towards global consensus on outcome measures for atopic eczema research: results of the HOME II meeting

The use of nonstandardized and inadequately validated outcome measures in atopic eczema trials is a major obstacle to practising evidence‐based dermatology. The Harmonising Outcome Measures for Eczema (HOME) initiative is an international multiprofessional group dedicated to atopic eczema outcomes research. In June 2011, the HOME initiative conducted a consensus study involving 43 individuals from 10 countries, representing different stakeholders (patients, clinicians, methodologists, pharmaceutical industry) to determine core outcome domains for atopic eczema trials, to define quality criteria for atopic eczema outcome measures and to prioritize topics for atopic eczema outcomes research. Delegates were given evidence‐based information, followed by structured group discussion and anonymous consensus voting. Consensus was achieved to include clinical signs, symptoms, long‐term control of flares and quality of life into the core set of outcome domains for atopic eczema trials. The HOME initiative strongly recommends including and reporting these core outcome domains as primary or secondary endpoints in all future atopic eczema trials. Measures of these core outcome domains need to be valid, sensitive to change and feasible. Prioritized topics of the HOME initiative are the identification/development of the most appropriate instruments for the four core outcome domains. HOME is open to anyone with an interest in atopic eczema outcomes research.

Scoring of atopic dermatitis by SCORAD using a training atlas by investigators from different disciplines

The ETAC (Early Treatment of the Atopic Child) study, a multi‐national double‐blind placebo‐controlled randomized trial, has been in progress since 1994. Fifty‐six centers in Europe and Canada participate in this study. A total of 817 children with atopic dermatitis [AD] were recruited. The severity of AD was scored using the SCORAD (objective criteria). Ninety‐eight investigators (mostly pediatricians) were trained by three members of the European Task Force on Atopic Dermatitis [ETFAD] to standardize their objective SCORAD scoring (system developed by the ETFAD). The experts selected photographs and prepared a training atlas. The percentages of photographs assessed by the 98 non‐expert investigators below, within and above the range of evaluations by the three experts were calculated. Taking over‐ and underscoring together, edema/papulation was the easie. st intensity item to score (82% within the range by the experts). The global symptom score, as well as lichenification. edema/papulation. oozing and excoriation registered by physicians with dermatological experience were not statistically significantly different from those by others. Erythema was statistically significantly better scored by those with dermatological experience. The results of the Euclidean Distance method showed that the item excoriations gave the largest distance. Erythema and excoriations were scored better by dermatologically experienced physicians (t‐test, p=0.042 and p=0.063 respectively), but lichenification was better scored by non‐dermatologically experienced physicians (p=0.013). The extent of surface area involved in the disease was calculated on 3 sets of photographs. Most evaluations by the 98 nonexpert investigators were within the range of the experts. Dermatologically experienced physicians scored significantly better than the others (Mest, p=0.006). This training program is useful for standardizing the scoring in AD and indicates that SCORAD can be used by investigators from different disciplines.

Propranolol for treatment of ulcerated infantile hemangiomas

BACKGROUND Ulcerated infantile hemangiomas (IH) are a therapeutic challenge. Propranolol, a nonselective beta-blocker, was recently introduced as a novel treatment for IH. OBJECTIVE To evaluate our experience of propranolol in the management of ulcerated IH. METHODS A national, multicenter, retrospective, observational study was conducted. Data were collected from the medical charts of patients treated from 2008 to 2009 and supplemented by information obtained from parents during targeted telephone interviews. RESULTS Thirty-three infants with propranolol-treated ulcerated IH were included. The average time to complete ulceration healing was 4.3 weeks in 30 of 33 patients and was significantly faster for head-and-neck locations (P = .0354). The mean time to complete pain control was 14.5 days. Parents rated treatment as very effective for 27 of 31 patients and very well tolerated for 29 of 31 cases. LIMITATIONS This was a retrospective uncontrolled study. CONCLUSION Propranolol appears to be an effective and well-tolerated treatment for ulcerated IH.

Report from the third international consensus meeting to harmonise core outcome measures for atopic eczema/dermatitis clinical trials (HOME)

This report provides a summary of the third meeting of the Harmonising Outcome Measures for Eczema (HOME) initiative held in San Diego, CA, U.S.A., 6–7 April 2013 (HOME III). The meeting addressed the four domains that had previously been agreed should be measured in every eczema clinical trial: clinical signs, patient‐reported symptoms, long‐term control and quality of life. Formal presentations and nominal group techniques were used at this working meeting, attended by 56 voting participants (31 of whom were dermatologists). Significant progress was made on the domain of clinical signs. Without reference to any named scales, it was agreed that the intensity and extent of erythema, excoriation, oedema/papulation and lichenification should be included in the core outcome measure for the scale to have content validity. The group then discussed a systematic review of all scales measuring the clinical signs of eczema and their measurement properties, followed by a consensus vote on which scale to recommend for inclusion in the core outcome set. Research into the remaining three domains was presented, followed by discussions. The symptoms group and quality of life groups need to systematically identify all available tools and rate the quality of the tools. A definition of long‐term control is needed before progress can be made towards recommending a core outcome measure.

Therapeutic Patient Education in Atopic Dermatitis: Worldwide Experiences

Therapeutic patient education (TPE) has proven effective in increasing treatment adherence and improving quality of life (QoL) for patients with numerous chronic diseases, especially atopic dermatitis (AD). This study was undertaken to identify worldwide TPE experiences in AD treatment. Experts from 23 hospitals, located in 11 countries, responded to a questionnaire on 10 major items. Patients in TPE programs were mainly children and adolescents with moderate to severe AD or markedly affected QoL. Individual and collective approaches were used. Depending on the center, the number of sessions varied from one to six (corresponding to 2 to 12 hours of education), and 20 to 200 patients were followed each year. Each centers education team comprised multidisciplinary professionals (e.g., doctors, nurses, psychologists). Evaluations were based on clinical assessment, QoL, a satisfaction index, or some combination of the three. When funding was obtained, it came from regional health authorities (France), insurance companies (Germany), donations (United States), or pharmaceutical firms (Japan, Italy). The role of patient associations was always highlighted, but their involvement in the TPE process varied from one country to another. Despite the nonexhaustive approach, our findings demonstrate the increasing interest in TPE for managing individuals with AD. In spite of the cultural and financial differences between countries, there is a consensus among experts to integrate education into the treatment of eczema.

Therapeutic patient education in children with atopic dermatitis: position paper on objectives and recommendations

Poor adherence is frequent in patients with atopic dermatitis (AD), leading to therapeutic failure. Therapeutic patient education (TPE) helps patients with chronic disease to acquire or maintain the skills they need to manage their chronic disease. After a review of the literature, a group of multispecialty physicians, nurses, psychologists, and patients worked together during two international workshops to develop common recommendations for TPE in AD. These recommendations were structured as answers to nine frequently asked questions about TPE in AD: What is TPE and what are its underlying principles? Why use TPE in the management of AD? Who should benefit from TPE in AD? How can TPE be organized for AD? What is the assessment process for TPE in AD? What is the evidence of the benefit of TPE in AD? Who are the people involved in TPE? How should TPE be funded in dermatology? What are the limits of the TPE process?

Executive dysfunction in children with neurofibromatosis type 1: a study of action planning.

In this study, we tested the hypothesis that action planning is impaired in children with neurofibromatosis type 1 (NF1). Thirty-six children with NF1 were pair-matched to 36 healthy controls (HC) on age (range, 7-12 years), sex, and parental education level, and both groups were administered three action-planning tasks. To examine the relation of task performance to attention deficit hyperactivity disorder (ADHD), the NF1 group was divided into subsets of children who met or did not meet criteria for ADHD. Children with NF1 performed less well than HC on all planning tasks, and differences remained when controlling for IQ or a measure of visuospatial skill. Both the NF1 with ADHD subset and NF1 without ADHD subset performed more poorly than HC on two of the tasks, whereas only the NF1 with ADHD subset performed worse than HC on the third planning task. The results underscore the importance of evaluating executive function in children with NF1 and suggest that deficits in this domain may be only partially related to ADHD. Planning deficits in children with NF1 may be part of their cognitive phenotype. Identifying these deficits is relevant in determining factors contributing to learning problems and in developing appropriate interventions.