ETFAD/EADV Eczema task force 2020 position paper on diagnosis and treatment of atopic dermatitis in adults and children.

Atopic dermatitis (AD) is a highly pruritic, chronic inflammatory skin disease. The diagnosis is made using evaluated clinical criteria. Disease activity and burden are best measured with a composite score, assessing both objective and subjective symptoms, such as SCORing Atopic Dermatitis (SCORAD). AD management must take into account clinical and pathogenic variabilities, the patient's age and also target flare prevention. Basic therapy includes hydrating and barrier-stabilizing topical treatment universally applied, as well as avoiding specific and unspecific provocation factors. Visible skin lesions are treated with anti-inflammatory topical agents such as corticosteroids and calcineurin inhibitors (tacrolimus and pimecrolimus), which are preferred in sensitive locations. Topical tacrolimus and some mid-potency corticosteroids are proven agents for proactive therapy, which is defined as the long-term intermittent anti-inflammatory therapy of frequently relapsing skin areas. Systemic anti-inflammatory or immunosuppressive treatment is a rapidly changing field requiring monitoring. Oral corticosteroids have a largely unfavourable benefit-risk ratio. The IL-4R-blocker dupilumab is a safe, effective and licensed, but expensive, treatment option with potential ocular side-effects. Other biologicals targeting key pathways in the atopic immune response, as well as different Janus kinase inhibitors, are among emerging treatment options. Dysbalanced microbial colonization and infection may induce disease exacerbation and can justify additional antimicrobial treatment. Systemic antihistamines (H1R-blockers) only have limited effects on AD-related itch and eczema lesions. Adjuvant therapy includes UV irradiation, preferably narrowband UVB or UVA1. Coal tar may be useful for atopic hand and foot eczema. Dietary recommendations should be patient-specific, and elimination diets should only be advised in case of proven food allergy. Allergen-specific immunotherapy to aeroallergens may be useful in selected cases. Psychosomatic counselling is recommended to address stress-induced exacerbations. Efficacy-proven 'Eczema school' educational programmes and therapeutic patient education are recommended for both children and adults.

ERCC2 mutations in two siblings with a severe trichothiodystrophy phenotype.

BACKGROUND: Trichothiodystrophy (TTD) describes a group of rare genetic disorders of DNA repair, characterized by sulphur-deficient hair, skin anomalies and systemic complications like preterm delivery, neurological impairment, haematological and ophthalmological abnormalities and life-threatening infections. OBJECTIVES: The aim of this case report was to investigate the contribution of the gene mutation to the phenotype. METHODS: We describe the clinical and molecular characteristics of a family with two TTD-affected siblings who died before the age of 2 years. RESULTS: The causal mutated gene is the ERCC2 gene, and one of the identified mutations is the c.2164C>T (p.Arg722Trp) variant. The association of this mutation with a severe TTD phenotype was suggested earlier in literature, and the present family adds further evidence to this hypothesis. CONCLUSION: Accurate identification of the underlying genetic defect can guide the clinical follow-up and counselling of patients and their families.

European task force on atopic dermatitis position paper: treatment of parental atopic dermatitis during preconception, pregnancy and lactation period.

Atopic dermatitis (AD) is a common inflammatory skin disease that affects both children and adults, including a large number of adults of reproductive age. Several guidelines for the treatment of AD exist, yet specific recommendations for the treatment of pregnant or lactating women and for adults planning to have a child are often lacking. This position paper from the European Task force on Atopic Dermatitis (ETFAD) is based on up-to-date scientific literature on treating pregnant and lactating women as wells as adults with AD planning to have a child. It is based on the expert opinions of members of the ETFAD and on existing safety data on the proposed treatments, many of which are derived from patients with other inflammatory diseases or from transplantation medicine. For treating future parents, as well as pregnant and lactating women with AD, the use of topical treatments including moisturizers, topical corticosteroids, tacrolimus, antiseptics such as chlorhexidine, octenidine, potassium permanganate and sodium hypochlorite (bleach) is deemed to be safe. Ultraviolet (UV) therapy may also be used. Systemic treatment should be prescribed only after careful consideration. According to the opinion of the ETFAD, treatment should be restricted to systemic corticosteroids and cyclosporine A, and, in selected cases, azathioprine.

Topical corticosteroid phobia in atopic dermatitis: International feasibility study of the TOPICOP score.

BACKGROUND: Adherence to topical corticosteroids (TCS) is essential for the effective treatment of atopic dermatitis but can be limited by concerns about their use. This study examined the feasibility of applying the validated TOPICOP score for assessing TCS phobia across different countries. METHODS: This was a prospective multicentre feasibility study conducted in 21 hospitals in 17 countries. Patients >3 months of age with atopic dermatitis or their parents or legal representatives completed a validated translation of the TOPICOP questionnaire in the country's native language. Respondents also completed questionnaires collecting opinions about the feasibility and acceptability of the TOPICOP questionnaire. RESULTS: A total of 1564 participants in 15 countries were included in the analysis. 81% of respondents considered the questions clear or very clear, and 79% reported that it took less than 5 minutes to complete. Each of the individual items in the TOPICOP questionnaire was considered to be not at all difficult to answer by 49% to 74% of participants. The mean global TOPICOP score was 44.7%±20.5. Mean TOPICOP subscores were 37.0±22.8% for knowledge and beliefs, 54.7±27.8% for fears and 50.1±29.1% for behaviours. Global scores and subscores differed between countries, although the subscores did not always vary in parallel, suggesting different levels of TCS phobia and different drivers for each country. CONCLUSIONS: The TOPICOP score can be feasibly applied across countries and may therefore be useful for obtaining qualitative and quantitative data from international studies and for adapting patient education and treatment.

Cochlear implants in Belgium: Prevalence in paediatric and adult cochlear implantation.

OBJECTIVES: Belgium, and especially the northern region called Flanders, has been a centre of expertise in cochlear implants and early hearing screening for many years. Cochlear implants are reimbursed by the Belgian National Institute for Health and Disability (BNIHD) Insurance in adults and in children since October 1994. More than 20 years later, we would like to measure the prevalence of cochlear implants in adults and in children till now. MATERIALS AND METHODS: Based on scientific research data on the prevalence of severe to profound hearing loss in adults and in children and on the number of implantations from the data of the BNIHD, we could measure the percentages of paediatric and adult CI users in comparing to the number of CI candidates. RESULTS: The degree of utilisation of cochlear implantation varies considerably between the paediatric and the adult population. On average, 78% of deaf children are receiving cochlear implants, but in adults only 6.6% of CI candidates are receiving one. CONCLUSION: There are big differences in Belgium in utilisation of cochlear implants between adults and children. Because of the underutilisation of cochlear implants, especially in adults, we have to work on raising the general awareness of the benefits of cochlear implants, and its improvement in quality of life, based on cost-effectiveness data and on guidelines for good clinical practice.

International survey of cochlear implant candidacy.

BACKGROUND: The goal of this work was to determine international differences in candidacy based on audiometric and speech perception measures, and to evaluate the information in light of the funding structure and access to implants within different countries. METHOD: An online questionnaire was circulated to professionals in 25 countries. There were 28 respondents, representing the candidacy practice in 17 countries. RESULTS: Results showed differences in the funding model between countries. Unilateral implants for both adults and children and bilateral implants for children were covered by national funding in approximately 60% of countries (30% used medical insurance, and 10% self-funding). Fewer countries provided bilateral implants routinely for adults: national funding was available in only 22% (37% used medical insurance and 41% self-funding). Main evolving candidacy areas are asymmetric losses, auditory neuropathy spectrum disorders and electro-acoustic stimulation. For countries using speech-based adult candidacy assessments, the majority (40%) used word tests, 24% used sentence tests, and 36% used a mixture of both. For countries using audiometry for candidacy (70-80% of countries), the majority used levels of 75-85 dB HL at frequencies above 1 kHz. The United Kingdom and Belgium had the most conservative audiometric criteria, and countries such as Australia, Germany, and Italy were the most lenient. Countries with a purely self-funding model had greater flexibility in candidacy requirements.

Patient-Oriented SCORAD (PO-SCORAD): a new self-assessment scale in atopic dermatitis validated in Europe.

BACKGROUND: Patient-oriented medicine is an emerging concept, encouraged by the World Health Organization, to greater involvement of the patient in the management of chronic diseases. The Patient-Oriented SCORing Atopic Dermatitis (PO-SCORAD) index is a self-assessment score allowing the patient to comprehensively evaluate the actual course of atopic dermatitis (AD), using subjective and objective criteria derived mainly from the SCORAD, a validated AD severity clinical assessment tool. OBJECTIVES: To validate the PO-SCORAD index in a large European population of patients exhibiting all forms of AD severity by assessing its correlation with the SCORAD index. PATIENTS/METHODS: Four hundred and seventy-one patients (185 adults, 286 children) consulting for AD in hospitals from 9 European countries were recruited. The investigators and the patients used the SCORAD and PO-SCORAD scales, respectively, to assess AD severity at inclusion (D0) and 28 ± 7 days later (D28). RESULTS: Patient-Oriented SCORing Atopic Dermatitis and SCORAD scores were significantly correlated at D0 [r = 0.67 (95% CI: 0.62; 0.72), P < 0.0001]. Consistency was confirmed at D28, with a stronger linear correlation between both scales [r = 0.79 (95% CI: 0.75; 0.83), P < 0.0001]. Absolute changes from baseline in SCORAD and PO-SCORAD scores were also significantly correlated [r= 0.71 (95% CI: 0.64; 0.76), P < 0.0001]. Although no specific intervention was investigated, AD improved over the study, with a decrease of PO-SCORAD and SCORAD scores from D0 to D28 by -19.19% and -24.39%, respectively. The consistency of the correlations was similar in the adult and children groups. CONCLUSIONS: This study validated the use of PO-SCORAD to self-assess AD severity and demonstrated its good correlation with SCORAD.

Bilateral versus unilateral cochlear implantation in young children.

OBJECTIVES: To compare the preverbal communication skills of two groups of young implanted children: those with unilateral implantation and those with bilateral implantation. MATERIAL AND METHODS: The study assessed 69 children: 42 unilaterally and 27 bilaterally implanted with age at implantation less than 3 years. The preverbal skills of these children were measured before and 1 year after implantation, using Tait Video Analysis that has been found able to predict later speech outcomes in young implanted children. RESULTS: Before implantation there was no significant difference between the unilateral group and the bilateral group. There was still no difference at 12 months following implantation where vocal autonomy is concerned, but a strongly significant difference between the groups for vocal turn-taking and non-looking vocal turns, the bilateral group outperforming the unilateral group. Regarding gestural turn-taking and gestural autonomy, there was a strongly significant difference between the two groups at the 12 month interval, and also a difference before implantation for gestural autonomy, the unilateral group having the higher scores. Multiple regression of non-looking vocal turns revealed that 1 year following implantation, bilateral implantation contributed to 51% of the variance (p<0.0001), after controlling for the influence of age at implantation and length of deafness which did not reach statistical significance. CONCLUSIONS: Profoundly deaf bilaterally implanted children are significantly more likely to use vocalisation to communicate, and to use audition when interacting vocally with an adult, compared with unilaterally implanted children. These results are independent of age at implantation and length of deafness.

Interpersonal conflicts at work as a predictor of self-reported health outcomes and occupational mobility.

OBJECTIVES: The aim of this prospective study was to examine the relationship between interpersonal conflicts at work and subsequent self-reported health outcomes (self-reported general health, need for recovery, and prolonged fatigue) and occupational mobility (internal mobility ie, changing job function, and external mobility ie, changing employers). METHODS: Data from the Maastricht Cohort Study on fatigue at work (n = 5582 for co-worker conflict; n = 5530 for supervisor conflict) were used. Interpersonal conflict with either co-workers or supervisors was assessed between baseline and 1-year follow-up. Outcomes were studied every 4 months between 1-year and 2-year follow-up. Logistic regression analyses using generalised estimating equations were conducted for each of the dichotomous outcomes, while controlling for demographic factors, the presence of a long-term illness, other workplace stressors, coping, and outcome at baseline. Analyses were conducted for men only. RESULTS: At baseline, conflicts with co-workers occurred in 7.2% of the study population, while conflicts with supervisors occurred in 9.5% of the study population. In general, this study showed that co-worker conflict was a statistically significant risk factor for the onset of an elevated need for recovery, prolonged fatigue, poor general health and external occupational mobility. Supervisor conflict was a significant risk factor for the onset of an elevated need for recovery, prolonged fatigue, external occupational mobility, and internal occupational mobility. CONCLUSIONS: The results of this study indicate a possible causal relationship between interpersonal conflicts at work and self-reported health and occupational mobility. Given the considerable impact of interpersonal conflicts at work on the individual worker and on the organisation, and the fact that interpersonal conflicts at work are highly prevalent, these findings underline the need for interventions aimed at preventing the occurrence of interpersonal conflicts at work, or at least reducing the harmful effects on both the employee and the organisation.

Trichothiodystrophy-like hair abnormalities in a child with keratitis ichthyosis deafness syndrome.

Keratitis ichthyosis deafness syndrome is a rare congenital ectodermal disorder. It appears to be genetically heterogeneous and may be caused by mutations in the connexin 26 (Cx26) gene (GJB2) or in the connexin 30 gene. It is characterized by the association of ichthyosis-like skin lesions, hearing loss, and vascularizing keratitis. We report the clinical and molecular findings in a 5-year-old girl with keratitis ichthyosis deafness syndrome. DNA sequencing in our patient revealed a p.Ser17Phe mutation in GJB2. Besides the typical clinical features of keratitis ichthyosis deafness syndrome, a peculiar intriguing finding not previously described in the literature in this condition was that polarizing light microscopy of the scalp hair in our patient revealed striking bright and dark bands as seen in trichothiodystrophy. Amino acid analysis of the hair sample also disclosed a reduced cysteine index. We emphasize that it would be of great benefit to examine hair shafts in other patients with keratitis ichthyosis deafness syndrome for trichothiodystrophy-like abnormalities.

Deaf children with cochlear implants before the age of 1 year: comparison of preverbal communication with normally hearing children.

OBJECTIVES: To compare preverbal behaviors of deaf children implanted under 1 year of age with age-matched hearing children. METHODS: The study assessed 20 children; 10 deaf children implanted under 1 year of age and 10 normally hearing children of the same age. Preverbal skills were measured before, 6 months, and 1 year after implantation, using Tait Video Analysis that is able to predict later speech outcomes in young implanted children. RESULTS: Regarding vocal turns, the normally hearing group outperformed the implanted group although the latter children became quite vocal, nearly 60% of their turns being taken in this way. The mean vocal autonomy in implanted children, 1 year after implantation, was very close to the respective of hearing children (38.5 versus 43.5). Regarding the non-looking vocal turns, by the 12-month interval, hearing children had somewhat higher scores than implanted children, but the difference was not significant and the increase in implanted children was much higher (40-fold increase versus 4-fold increase). However, implanted children were more likely to use silent communication than hearing children, although gestural turns were decreasing with time. CONCLUSIONS: The small numbers in this study, although two of the largest European cochlear implant centers were combined to recruit such young implantees, led us to be cautious in interpreting the results. However, it seems that in deaf implanted children under 1 year of age, some preverbal communication behaviors are developing to an extent (although at a somewhat lower level) not significantly different from those of age-matched normally hearing children.

Distinct phenotypic changes between the superficial and deep component of giant congenital melanocytic naevi: a rationale for curettage.

BACKGROUND: Giant congenital melanocytic naevi (GCMN) convey a 14-fold increased melanoma risk. In contrast, medium congenital melanocytic naevi (MCMN) are rarely associated with malignant transformation. Management of patients with GCMN is challenging and there is no consensus on the most appropriate strategy for treating these patients. OBJECTIVES: To provide a rationale for performing curettage of GCMN in the neonatal period in order to reduce the risk of malignant transformation to melanoma. METHODS: Twenty-six infants with GCMN who underwent biopsies before excisional surgery (n = 7) or curettage (n = 19) during the past 14 years (Academic Hospital, Vrije Universiteit Brussel) and 10 MCMN patients who underwent excision biopsies (Radboud University Nijmegen Medical Centre) were included in this study. Using these biopsies, we performed genetic and detailed immunohistochemical evaluations of changes that are associated with malignant transformation. Variables of interest included melanoma-associated BRAF mutations, proliferative activity, vascularity, cellular context and extracellular matrix architecture. RESULTS: GCMN and MCMN did not show oncogenic BRAF mutation and displayed similar features with respect to the amount of nonmelanocytic cells within the naevus and matrix architecture. Naevus cells in the superficial component of the GCMN, however, were more proliferative, and this component was more vascular compared with its deep component and with MCMN. In this study, none of the 19 newborn patients who underwent curettage developed a melanoma within a mean follow-up time of 7 years. CONCLUSIONS: The data presented here support the idea that curettage of GCMN in neonates has the potential for lowering the risk of developing cutaneous melanoma by not only obtaining an important numerical reduction of naevus cells but also removing the 'active' melanocytes.

The prevalence of positive reactions in the atopy patch test with aeroallergens and food allergens in subjects with atopic eczema: a European multicenter study.

BACKGROUND: The atopy patch test (APT) was proposed to evaluate IgE-mediated sensitizations in patients with atopic eczema (AE). OBJECTIVE: The prevalence and agreement with clinical history and specific IgE (sIgE) of positive APT reactions was investigated in six European countries using a standardized method. METHODS: A total of 314 patients with AE in remission were tested in 12 study centers on clinically uninvolved, non-abraded back skin with 200 index of reactivity (IR)/g of house dust mite Dermatophagoides pteronyssinus, cat dander, grass, and birch pollen allergen extracts with defined major allergen contents in petrolatum. Extracts of egg white, celery and wheat flour with defined protein content were also patch tested. APT values were evaluated at 24, 48, and 72 h according to the European Task Force on Atopic Dermatitis (ETFAD) guidelines. In addition, skin-prick test (SPT) and sIgE and a detailed history on allergen-induced eczema flares were obtained. RESULTS: Previous eczema flares, after contact with specific allergens, were reported in 1% (celery) to 34% (D. pteronyssinus) of patients. The frequency of clear-cut positive APT reactions ranged from 39% with D. pteronyssinus to 9% with celery. All ETFAD intensities occured after 48 and 72 h. Positive SPT (16-57%) and elevated sIgE (19-59%) results were more frequent. Clear-cut positive APT with all SPT and sIgE testing negative was seen in 7% of the patients, whereas a positive APT without SPT or sIgE for the respective allergen was seen in 17% of the patients. APT, SPT and sIgE results showed significant agreement with history for grass pollen and egg white (two-sided Pr > /Z/ < or = 0.01). In addition, SPT and sIgE showed significant agreement with history for the other aeroallergens. With regard to clinical history, the APT had a higher specificity (64-91% depending on the allergen) than SPT (50-85%) or sIgE (52-85%). Positive APT were associated with longer duration of eczema flares and showed regional differences. In 10 non-atopic controls, no positive APT reaction was seen. CONCLUSION: Aeroallergens and food allergens are able to elicit eczematous skin reactions after epicutaneous application. As no gold standard for aeroallergen provocation in AE exists, the relevance of aeroallergens for AE flares may be evaluated by APT in addition to SPT and sIgE. The data may contribute to the international standardization of the APT.

Medical authority and nursing integrity.

This paper explores the respective legitimacy or illegitimacy of medical authority over nursing work. The analysis makes use of Joseph Raz's ideas concerning the nature of authority. Various scenarios are considered which lend themselves to differing interpretations, and the conclusion reached is that acting in accordance with legitimate medical authority enhances rather than compromises the nurse's professional integrity. Difficulties, however, may lie in disentangling legitimate from illegitimate attempts to control nursing work.