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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. [ABSTRACT FROM AUTHOR]

Objectives: International guidelines and clearly defined care pathways on adult cochlear implantation (CI) are limited, and country-specific guidelines vary and are associated with disparate levels of access and systemic underuse across the world. In 2020, consensus statements were published representing the first step toward the development of international guidelines on best practices for CI in adults with severe to profound sensorineural hearing loss (SPNHL). The standard of care for adults with hearing loss should include treatments that best improve the individual's quality of life through optimizing hearing function, social participation, and engagement. For adults with SPNHL, the standard of care includes an accurate diagnosis and timely referral to an appropriate specialist centre for assessment and counselling. When it is indicated as the most beneficial treatment option, the patient should be advised by an appropriate healthcare professional about access to cochlear implantation (CI) and aftercare. Material and methods: An international group of CI users and experts in the fields of otology, audiology, and hearing care have been brought together to form a Task Force in partnership with the Cochlear Implant International Community of Action (CIICA). The aim of the Task Force is to develop living practice guidelines and guidance that can be adapted and adopted in country, in order to optimise the care for adults indicated for CI. Member affiliations will extend to national and international organisations and a wide range of stakeholders implementing hearing care solutions within the community and most importantly patient representatives with SPSNHL. Objective: The overall objective of the Task Force is to contribute to and support the effective development and subsequent dissemination and adoption into practice of a set of accurate, consistent, and usable guidance and guidelines. Results: As the guidance and guidelines will need to be updated as new evidence is published, the aim of the Task Force is therefore for continuity and evolution over the long-term. Developing a consistent approach to optimizing the care for hearing impaired adults who may not receive adequate benefit from hearing aids will help raise awareness and better de- fine referral and treatment pathways, so patients can receive information about a treatment option that may help them. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Otoferlin is a calcium sensor protein expressed in the inner hair cells and is important for proper synaptic transmission between inner hair cells and the afferent fibers of the spiral ganglion. Biallelic loss of function mutations in the OTOF gene lead to congenital severe-to-profound auditory neuropathy in both humans and in mice. These mutations are believed to be causal in 2--3% of individuals born with hearing loss. Infants with biallelic OTOF mutations are currently managed with assistive devices, but several groups are developing AAV-based gene therapies to address this population. Methods: We have developed DB-OTO, an Adeno-Associated Virus (AAV)-based gene transfer therapy for rescue of hearing in Otoferlin deficiency. DB-OTO expresses a corrected OTOF cDNA from a hair cell-specific Myo15 promoter and is delivered using an AAV1-based capsid. Because human OTOF cDNA exceeds the packaging capacity of a standard AAV, DBOTO uses a dual AAV system to reconstitute the full-length OTOF coding sequence. Results: We have previously shown that DB-OTO can rescue hearing function in OTOFQ828X/ Q828X mutant mice as measured by ABR, that it can be successfully delivered to the primate ear via RW infusion with vestibular fenestration, and that using a cell-specific promoter is key to its function. Here, in preparation for the initiation of clinical studies, we further characterized the dose translatability of DB-OTO between mice and non-human primates. To better characterize the dose-response of DB-OTO, we dosed mice over a >10 dose range and evaluated their hearing recovery over several months. We found that at higher doses, tone-burst response in previously deaf OTOFQ828X/Q828X mice extends to the apex of the cochlea, whereas with low doses, the best recovery is seen around 22 kHz. We also treated OTOFwt/wt mice to characterize the tolerability of DB-OTO over the same dose range. Using RT-PCR, we showed that DBOTO expression increases continuously for the first several weeks after dosing in mice, consistent with functional recovery. In parallel, we evaluated the expression timing in nonhuman primates using the same assay. There, the expression appeared to plateau in a comparable timeframe following dosing. Using this assay, DB-OTO expression levels at plateau in mice and primates were compared and used to confirm prior assumptions about dose translation based on expression from surrogate vectors. Together, these data support and inform our plans for clinical translation of DB-OTO. Conclusions: DBOTO is a promising emerging therapeutic for genetic hearing loss and has the potential to provide the first clinical proofof- concept for gene therapy in the inner ear. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to measure the number of paediatric CI candidates in new-borns with an asymmetric hearing loss with one deaf ear. We also want to compare the number of candidates with the real number of implantations in case of asymmetric hearing loss with one deaf ear. Material and methods: Flanders, the north part of Belgium, was in 1998 the first region in Europe to implement Universal Neonatal Hearing Screening (UNHS). During the period 1998--2016 (18 years) more the 1.1 million ears were screened and in 2445 baby's a permanent hearing loss was detected. Based on these data we had a clear overview on the expected number of cochlear implants in cases with asymmetric hearing loss. Since March 2015, paediatric cochlear implantation in case of asymmetric hearing loss is reimbursed in Belgium for children younger than age 12, who have a hearing loss of >85 dB in one ear and >60 dB in the other ear. The implantation also has to be done within 3 years after reaching these criteria. These expected data were compared with the actual number of CI's in cases with asymmetric hearing loss in 2017 and 2018. Results: Looking at the data from the UNHS carried out by the government of health care during the period 1998--2016, 2445 baby's had a permanent hearing loss. Of these baby's 1172 (48%) had a bilateral symmetric hearing loss, 736 (30%) an unilateral hearing loss and 535 (22%) had a bilateral asymmetric hearing loss. Of the group of baby's with an asymmetric hearing loss 181 (7.4%) had one deaf ear and 118 (4.8%) baby's fall within the Belgium CI-criteria. This means we can expect a yearly number of 12.5 new-borns with an asymmetric hearing loss and one deaf ear (or 1/12500 new-borns) of which 6.6 (52.8%) fall within the current Belgian criteria. But looking at the current number of asymmetric implantations in Belgium, we see that 28 children with an asymmetric hearing loss have received a CI in 2018. If only 6.6 of this group are new-borns with an asymmetric hearing loss, it means that 4 times more children in the asymmetric group were not candidates at birth, but have developed a progressive hearing loss in the early years. Conclusions: vLooking at the population of new-borns, we can expect that 1/12500 new-borns have an asymmetric hearing loss with one deaf ear and falls within the candidacy for cochlear implantation. But 3 times more children develop a progressive asymmetric hearing loss early in life, which means we have to multiply the new-born candidates with a factor 3 to estimate the real number of asymmetric paediatric CI candidates. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Summary: 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. [Copyright &y& Elsevier]

OBJECTIVE:: To explore a possible causal relationship between psychosocial work characteristics and mental health. METHODS:: Using longitudinal data from the Maastricht Cohort Study on 'Fatigue at Work' (n = 2332), the effects of changes in job demands and decision latitude on subsequent changes in need for recovery and prolonged fatigue were studied. RESULTS:: Increasing job demands are a significant predictor of a subsequent increase in need for recovery (beta = 0.063) and prolonged fatigue (beta = 0.057). An increase in decision latitude predicted a subsequent decrease in need for recovery (beta = -0.078) and prolonged fatigue (beta = -0.063). After adjusting for changes in other work characteristics, the effects on changes in prolonged fatigue were no longer significant. CONCLUSION:: These findings support a possible causal relationship between work characteristics and mental health and can be used for designing effective prevention and intervention strategies. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

This article examines one aspect of the criticism of inauthenticity that can be levelled against the trustworthiness of professional relationships in general and nurse-patient relationships in particular. The overall question is: are such relationships inherently trustworthy or untrustworthy, from the patient's point of view? The author concludes that, in spite of legitimate grounds for concern, and while it remains true that nurse-patient relationships may be untrustworthy, they are not inherently so for reasons of inauthenticity relating to emotional labour. The arguments used to defend this claim take their force from the idea that different criteria may be needed to assess the authenticity of nurse-patient relationships from those used to evaluate authenticity in ordinary social relationships. The utility of Hochschild's idea of 'deep' acting, as offering a useful model for the management of emotions in nursing, is examined and rejected. [ABSTRACT FROM AUTHOR]

This paper explores the nature of trust in nurse-patient relationships from the perspective of the patient's trust in the nurse and what might be said to then render such a relationship trustworthy, from the patient's point of view. The paper commences with a general examination of the nature of trust, followed by consideration of the nature of professional-patient relationships in healthcare, with emphasis on nurse- patient relationships in particular. The nature of this relationship is used to provide grounds for arguing, both morally and conceptually, that the nature of patients trust in those who nurse them is generally such that for the nurse to respond in a trustworthy way, she must care about her patients, not just for them. In arriving at this conclusion, the paper deliberately restricts its focus to nurse-patient encounters where patients are adults and a relationship, as opposed to a brief engagement between two people, can be said to have taken place. The context for this focus is public sector health care in the United Kingdom. In the text, gendered pronouns referring to doctors, nurses and patients are meant to be inclusive of both sexes. [ABSTRACT FROM AUTHOR]

Objectives: The aim of the study was to find out the most important factors influencing complex language development and school performance in uni and bilateral implanted deaf children. Materials and Methods: We started up a retrospective study on 37 early screened (< 3 months) and early implanted (< age 3 y) deaf children: 12 are bilateral CI users, 9 are bimodal users (CI+HA) and 16 are unilateral CI-users. All children received support and habilitation from the same centre. Two groups of children (unilateral and bilateral device users) were compared (on the short and long term) concerning vocabulary, speech perception in quit and in complex listening situations, verbal cognition and school performance (on literacy). Results:A significant effect of speech perception abilities in complex listening situations (perception of soft speech at 45 dB SPL and of speech in noise) on verbal intelligence and on literacy performance was found. This suggests that bilateral input and the possibility to come to incidental language learning was the underlying reason for improved speech perception. These abilities enable at least some of the children to develop age-equivalent verbal cognition skills and school performance. Conclusion: Deaf children who use bilateral devices have the opportunity to develop good speech perception skills in complex listening conditions which enables at least some of the children to develop age-equivalent verbal cognition skills and school performance. But there is huge variability in outcomes, and in our presentation we will focus on how we have to adapt our habilitation and education to these outcomes. [ABSTRACT FROM AUTHOR]

Lipoid proteinosis is a very rare autosomal recessive disorder that has been shown to result from mutations in the ECM1 gene. It is characterized by deposition of hyaline material in virtually any organ in the body, but particularly the skin, pharyngeal mucosa and the larynx. Typical clinical symptoms are hoarseness, marked vulnerability of the skin with scarring, and beading of papules on the eyelids. We report a 6-year-old boy born from consanguineous parents. He suffered from recurrent aphtae and easily damaged skin with poor wound healing. On clinical examination he had pox-like scars on the skin of the trunk and limbs, beaded papules on the eyelids and a hoarse voice. Electron microscopy of the skin revealed thickened vessel walls in the reticular dermis with reduplication of basal membranes, and an electron-dense microgranular deposit containing occasional collagen fibers around the blood vessels. Mutation analysis by sequencing the 10 exons and parts of the flanking introns of the ECM1 gene showed a homozygous mutation in the acceptor splice site of intron 8, predicting the loss of exon 9 from the transcript, and protein. Discussion. Lipoid proteinosis is typically diagnosed by its clinical dermatological manifestations, especially the pathognomonic ‘string of beads’ on the eyelids. Electron microscopy may be used for diagnosis in young children when pathognomonic lesions are not yet present. The discovery of the mutations of the ECM1 gene allows the use of genetic testing to confirm the initial clinical diagnosis. [ABSTRACT FROM AUTHOR]

Giant congenital melanocytic naevi (GCMN) are disfiguring lesions with an increased malignancy risk. Since 13 years we manage these lesions by curettage in the neonatal period in order to obtain an acceptable cosmetic result and to decrease the psychosocial inconvenience to the patient. From the curetted material we studied the in vitro characteristics of these GCMN cells in standardized culture conditions and compared them with the characteristics of normal and malignant melanocytes. Our preliminary results show that the supernatants of the GCMN cells contains stimulatory factors which increase cellular growth. bFGF and EGF were found to be able to stimulate the cellular growth of the GCMN cell lines, but could not be detected in the conditioned media of the GCMN cells. MMP-2 and MMP-9 were not detected in the GCMN cells and the fibroblasts from GCMN but MMP-2 was detected in the HBL melanoma cells. E-cadherin expression in the GCMN cells seems to be the same as that in normal melanocytes, but twofold higher than in the HBL melanoma cells. [ABSTRACT FROM AUTHOR]

Abstract: 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. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Atopic dermatitis ( AD) is a clinically defined, highly pruritic, chronic inflammatory skin disease of children and adults. The diagnosis is made using evaluated clinical criteria. Disease activity is best measured with a composite score assessing both objective signs and subjective symptoms, such as SCORAD. The management of AD must consider the clinical and pathogenic variabilities of the disease and also target flare prevention. Basic therapy includes hydrating topical treatment, as well as avoidance of specific and unspecific provocation factors. Anti-inflammatory treatment of visible skin lesions is based on topical glucocorticosteroids and the topical calcineurin inhibitors tacrolimus and pimecrolimus. Topical calcineurin inhibitors are preferred in sensitive locations. Tacrolimus and mid-potent steroids are proven for proactive therapy, which is long-term intermittent anti-inflammatory therapy of the frequently relapsing skin areas. Systemic anti-inflammatory or immunosuppressive treatment is indicated for severe refractory cases. Biologicals targeting key mechanisms of the atopic immune response are promising emerging treatment options. Microbial colonization and superinfection may induce disease exacerbation and can justify additional antimicrobial treatment. Systemic antihistamines (H1R-blockers) may diminish pruritus, but do not have sufficient effect on lesions. Adjuvant therapy includes UV irradiation, preferably UVA1 or narrow-band UVB 311 nm. 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. 'Eczema school' educational programmes have been proven to be helpful for children and adults. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The atopy patch test (APT) was proposed to evaluate IgE-mediated sensitizations in patients with atopic eczema (AE).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.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 miteDermatophagoides 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.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% withD. pteronyssinusto 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| ≤ 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.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. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which discuses the case of two Moroccan sisters who were both treated for multiple cutaneous spinocellular carcinomas (SCC) and basal cell carcinomas (BCC) and diagnosed with Xeroderma pigmentosum (XP) and leukemia.