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BACKGROUND: Lipoid proteinosis (LP), also known as Urbach-Wiethe disease, is a rare autosomal recessive genodermatosis, caused by mutations in the ECM1 gene. This results in the deposition of periodic acid-Schiff (PAS)-positive, hyaline-like material on the skin, mucosae and internal organs. OBJECTIVES: To present a case report of LP and a systematic review to synthesize the scientific literature on the management of this uncommon and frequently missed diagnosis. METHODS: We present a case report of a 48-year-old man with LP who exhibited significant improvement after oral acitretin therapy. To address the lack of large case-control studies on LP treatment, we performed a systematic review of the literature following the PRISMA 2020 criteria. The search was conducted in PubMed, Web of Science, Cochrane and Scopus databases from inception until June 2023. To assess the methodological quality of case reports and case series, we used the Joanna Briggs Collaboration critical appraisal tool. RESULTS: We included 25 studies that met eligibility criteria. Data from 44 patients with a histopathologically confirmed diagnosis were analysed. Treatment ranged from systemic therapies (acitretin, etretinate, dimethyl sulfoxide, corticosteroids, penicillamine) to surgical or laser procedures. Regarding methodological quality, the main discrepancies arose in the reporting of participant characteristics and treatment interventions. CONCLUSIONS: Low-dose oral acitretin could have potential in managing LP, exhibiting fewer side-effects compared with other therapeutic agents. Further research is needed to establish more comprehensive and evidence-based treatment guidelines.
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Acitretina , Proteinosis Lipoidea de Urbach y Wiethe , Humanos , Proteinosis Lipoidea de Urbach y Wiethe/genética , Proteinosis Lipoidea de Urbach y Wiethe/patología , Proteinosis Lipoidea de Urbach y Wiethe/tratamiento farmacológico , Proteinosis Lipoidea de Urbach y Wiethe/diagnóstico , Masculino , Acitretina/uso terapéutico , Persona de Mediana Edad , Queratolíticos/uso terapéutico , Resultado del TratamientoRESUMEN
BACKGROUND: Sleep disturbance is a prominent symptom of atopic dermatitis (AD) and can result in insomnia, daytime fatigue, drowsiness, reduced productivity and impaired quality of life (QoL). OBJECTIVES: The Dupilumab Effect on Sleep in AD Patients (DUPISTAD) phase IV randomized double-blinded placebo-controlled study evaluated the impact of dupilumab treatment on sleep and other patient- and physician-reported outcomes. METHODS: Adults with moderate-to-severe AD were randomized 2 : 1 to dupilumab 300â mg once every 2 weeks (q2w) or placebo for 12 weeks; concomitant topical corticosteroids were permitted. Patients subsequently entered an open-label phase and received dupilumab 300â mg q2w for a further 12 weeks. The primary endpoint was the percentage change in sleep quality from baseline to week 12, assessed using a novel numeric rating scale (NRS). Secondary and exploratory endpoints included percentage change in peak pruritus NRS (PP NRS), change in SCORing Atopic Dermatitis (SCORAD), SCORAD sleep visual analogue scale (VAS), Eczema Area and Severity Index, Patient-Reported Outcomes Measurement Information System (PROMIS) sleep-related impairment T-score and the Epworth Sleepiness Scale. Sleep diary and wrist actigraphy measurements were recorded throughout the study. RESULTS: In total, 127 patients received dupilumab and 61 patients received placebo. Demographic and baseline disease characteristics were balanced between groups. Sleep quality NRS significantly improved in patients treated with dupilumab by week 12 vs. placebo [least squares mean of the difference (LSMD) -15.5%, P < 0.001]. PP NRS (LSMD -27.9%, P < 0.001), SCORAD (LSMD -15.1, P < 0.001), SCORAD sleep VAS (LSMD -2.1, P < 0.001) and PROMIS T-score (LSMD -3.6, P < 0.001) were also significantly improved at week 12 with dupilumab vs. placebo. The overall percentage of patients reporting treatment-emergent adverse events was lower in the dupilumab group (56.7%) than in the placebo group (67.2%). CONCLUSIONS: Dupilumab significantly improved sleep quality and perception of sleep continuity, itch, metrics of AD severity and QoL in adults with moderate-to-severe AD, with an acceptable safety profile compared with placebo.
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Dermatitis Atópica , Adulto , Humanos , Anticuerpos Monoclonales/efectos adversos , Dermatitis Atópica/complicaciones , Dermatitis Atópica/tratamiento farmacológico , Método Doble Ciego , Inyecciones Subcutáneas , Prurito/etiología , Prurito/inducido químicamente , Calidad de Vida , Índice de Severidad de la Enfermedad , Sueño , Resultado del TratamientoRESUMEN
OBJECTIVE: To analyse time trends in systemic lupus erythematosus (SLE) mortality and explore possible provincial clustering of SLE mortality in Spain (2001-2020). METHODS: We conducted an ecological study using deaths registered in SLE at the Spanish National Institute of Statistics between 2001 and 2020. Jointpoint regression models have been used to evaluate temporal trends. To analyse the spatial pattern of SLE mortality in men and women in Spain, crude rates, age-standardised mortality rates (ASMRs), smooth relative risk (RR) and posterior probabilities (PP) for RR greater than one for the period 2001-2020 were calculated. The Global Moran I index was used to assess the existence of global spatial autocorrelation. Local indicators of spatial association (LISA) and Kulldorff's spatial scan statistic were used to identify clusters. RESULTS: Over the 20 years analyzed in this study, the SLE average ASMR for the period was 2.7 for women and 0.7 for men, with a sex ratio (female/male) of 3.8. In men, no province showed a RR>1. Conversely, in women, eight provinces showed values of RR> 1 with a PP greater than 0.8 (Seville, Cadiz, Huelva and Murcia in the south, Barcelona, Zaragoza, Huesca and Leon in the north). In men, neither of the two methods detected a clustering of provinces. However, in women, both methods identified a cluster of provinces located in the southwest of the country (Huelva, Cádiz, Seville and Malaga) as a cluster with significant excess mortality. In the second cluster (centred on the province of Huelva) obtained with the Kulldorff method, two more provinces were added (Badajoz and Cordoba, also located in the southwest). CONCLUSIONS: We detected a cluster of provinces with an excess risk of female SLE mortality in the southwest of Spain.
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Lupus Eritematoso Sistémico , Humanos , Masculino , Femenino , España/epidemiología , Análisis por ConglomeradosRESUMEN
BACKGROUND: The distinction between in situ melanoma (MIS) and invasive melanoma is challenging even for expert dermatologists. The use of pretrained convolutional neural networks (CNNs) as ancillary decision systems needs further research. AIM: To develop, validate and compare three deep transfer learning (DTL) algorithms to predict MIS vs. invasive melanoma and melanoma with a Breslow thickness (BT) of < 0.8â mm vs. ≥ 0.8â mm. METHODS: A dataset of 1315 dermoscopic images of histopathologically confirmed melanomas was created from Virgen del Rocio University Hospital and open repositories of the International Skin Imaging Collaboration archive and Polesie S et al. (Dermatol Pract Concept 2021; 11:e2021079). The images were labelled as MIS or invasive melanoma and < 0.8â mm or ≥ 0.8â mm of BT. We conducted three trainings, and overall means for receiver operating characteristic (ROC) curves, sensitivity, specificity, positive and negative predictive value, and balanced diagnostic accuracy outcomes were evaluated on the test set with ResNetV2, EfficientNetB6 and InceptionV3. The results of 10 dermatologists were compared with the algorithms. Grad-CAM gradient maps were generated, highlighting relevant areas considered by the CNNs within the images. RESULTS: EfficientNetB6 achieved the highest diagnostic accuracy for the comparison between MIS vs. invasive melanoma (61%) and BT < 0.8â mm vs. ≥ 0.8â mm (75%). For the BT comparison, ResNetV2 with an area under the ROC curve of 0.76 and InceptionV3 with an area under the ROC curve of 0.75, outperformed the results obtained by the dermatologist group with an area under the ROC curve of 0.70. CONCLUSION: EfficientNetB6 recorded the best prediction results, outperforming the dermatologists for the comparison of 0.8â mm of BT. DTL could be an ancillary aid to support dermatologists' decisions in the near future.
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Melanoma , Neoplasias Cutáneas , Humanos , Dermatólogos , Dermoscopía/métodos , Neoplasias Cutáneas/diagnóstico , Melanoma/diagnóstico , Algoritmos , Aprendizaje Automático , Melanoma Cutáneo MalignoRESUMEN
BACKGROUND: Tralokinumab was recently approved for the treatment of moderate-to-severe atopic dermatitis (AD) and is the first selective interleukin (IL)-13 inhibitor that specifically neutralizes IL-13 with high affinity. OBJECTIVES: To determine the real-life short-term effectiveness and safety of tralokinumab treatment in patients with moderate-to-severe AD. METHODS: A multicentre retrospective study was conducted including adult patients with moderate-to-severe AD who started tralokinumab treatment from 1 April to 30 June 2022 in 16 Spanish hospitals. Demographic and disease characteristics, severity and quality of life scales were collected at the baseline visit and at weeks 4 and 16. RESULTS: Eighty-five patients were included. Twenty-seven patients (32%) were non-naive to advanced therapy (biological or Janus kinase inhibitors inhibitors). All included patients had severe disease with baseline Eczema Area and Severity Index (EASI) scores of 25.4 (SD 8.1), Dermatology Life Quality Index (DLQI) 15.8 (5.4) and peak pruritus numerical rating scale (PP-NRS) 8.1 (1.8) and 65% had an Investigator's Global Assessment (IGA) of 4. At week 16, there was improvement on all scales. The mean EASI decreased to 7.5 (SD 6.9, 70% improvement), SCORing Atopic Dermatitis improved 64% and PP-NRS, 57%. Also, 82%, 58% and 21% of the patients achieved EASI 50, 75 and 90, respectively. The percentage of EASI 75 responders was significantly higher among the naive vs. non-naive groups (67% vs. 41%). The safety profile was acceptable. CONCLUSIONS: Patients, with a long history of disease and prior multidrug failure, showed a good response to tralokinumab, confirming clinical trial results.
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Dermatitis Atópica , Adulto , Humanos , Dermatitis Atópica/tratamiento farmacológico , Calidad de Vida , Estudios Retrospectivos , Resultado del Tratamiento , Prurito/tratamiento farmacológico , Índice de Severidad de la Enfermedad , Método Doble CiegoRESUMEN
BACKGROUND: Systemic sclerosis (SSc) is an autoimmune chronic rheumatic disease with a high mortality rate, which continues to be a challenge for clinicians today. AIM: To assess changes in mortality trends in the Spanish SSc population between 1980 and 2019, taking into account the independent effects of sex, age, time period and birth cohort. METHODS: SSc death records and mid-year population data were collected from the National Statistics Institute. Age-standardized mortality rates were calculated for the overall population and for each sex (male, female) and age group (5-year groups). Significant changes in mortality trends were identified by joinpoint regressions. An age-period-cohort (APC) analysis and potential years of life lost (PYLL) analysis were performed to identify the burden of SSc. RESULTS: Age-standardized mortality rates due to SSc increased from 1.87 (95% CI 1.00-3.02) per 1 000 000 inhabitants between 1980 and 1984, to 2.47 (95% CI 1.74-3.02) per 1 000 000 inhabitants between 2015 and 2019. The relative risk of mortality fell in all groups in cohorts born after 1990. The PYLL rates showed a gradual rise for both sexes. CONCLUSION: There was an increase in overall SSc mortality in Spain during the 39 years evaluated, although there was a progressive drop for men.
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Esclerodermia Sistémica , Humanos , Masculino , Femenino , España/epidemiología , Estudios de CohortesRESUMEN
OBJECTIVE: To analyse mortality rate trends in Spain for oral cavity and oropharyngeal cancer (OCOPC) from 1979 to 2018, evaluating differences between oral cavity cancer (OCC) and oropharyngeal cancer (OPC). MATERIALS AND METHODS: Death certificates and mid-year population data were collected from the Spanish National Statistics Institute. Age-standardized mortality rates were calculated using the direct method. Joinpoint regressions were used to identify significant changes in mortality trends. Independent effects of age, period and cohort (APC) were estimated. RESULTS: A total of 52,057 deaths were registered from OCOPC, 38,988 from OCC and 13,069 from OPC between 1979 and 2018. While OCC mortality rates declined, OCOPC rates increased slightly and OPC significantly. OCC and OPC mortality reached their highest values between 1979 and 1992, when OCC rates began to decrease in males and OPC levelled off until 2018. Lip cancer suffered the highest drop. APC models showed a mortality increase in males and females from 40 to 45 and 50 to 55 years of age, respectively. CONCLUSIONS: Favourable OCC mortality trends was plausibly influenced by decreased tobacco/alcohol consumption, while OPC rise was probably associated with increased human papillomavirus infection. The importance of closely monitoring these cancers by age group, sex and location, and continuing with preventive measures against known risk factors, is highlighted.
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Neoplasias de la Boca , Neoplasias Orofaríngeas , Infecciones por Papillomavirus , Femenino , Humanos , Incidencia , Masculino , Neoplasias Orofaríngeas/epidemiología , Infecciones por Papillomavirus/epidemiología , España/epidemiologíaRESUMEN
BACKGROUND: Digital strategies are innovative approaches to the prevention of skin cancer, but the attrition following this kind of intervention needs to be analyzed. OBJECTIVE: The aim of this paper is to assess the dropouts from studies focused on digital strategies for the prevention of skin cancer. METHODS: We conducted this systematic review with meta-analyses and metaregression according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statements. Search terms for skin cancer, digital strategies, and prevention were combined to search PubMed, Scopus, Web of Science, CINAHL, and Cochrane Library from inception until July 2022. Randomized clinical trials that reported dropouts of participants and compared digital strategies with other interventions to prevent skin cancer in healthy or disease-free participants were included. Two independent reviewers extracted data for analysis. The Revised Cochrane Collaboration Bias tool was employed. We calculated the pooled dropout rate of participants through a meta-analysis of proportions and examined whether dropout was more or less frequent in digital interventions against comparators via an odds ratio (OR) meta-analysis. Data were pooled using a random-effects model. Subgroup meta-analyses were conducted in a meta-analysis of proportions and OR meta-analysis to assess the dropout events when data were sorted by digital interventions or control comparator. A univariate metaregression based on a random-effects model assessed possible moderators of dropout. Participants' dropout rates as pooled proportions were calculated for all groups combined, and the digital and comparator groups separately. OR>1 indicated higher dropouts for digital-based interventions. Metaregressions were performed for age, sex, length of intervention, and sample size. RESULTS: A total of 17 studies were included. The overall pooled dropout rate was 9.5% (95% CI 5.0-17.5). The subgroup meta-analysis of proportions revealed a dropout rate of 11.6% for digital strategies (95% CI 6.8-19.0) and 10.0% for comparators (95% CI 5.5-17.7). A trend of higher dropout rates for digital strategies was observed in the overall (OR 1.16, 95% CI 0.98-1.36) and subgroup OR meta-analysis, but no significant differences were found between the groups. None of the covariates moderated the effect size in the univariate metaregression. CONCLUSIONS: Digital strategies had a higher dropout rate compared to other prevention interventions, but the difference was not significant. Standardization is needed regarding reporting the number of and reasons for dropouts. TRIAL REGISTRATION: International Prospective Register of Systematic Reviews (PROSPERO) CRD42022329669; https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=329669.
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Neoplasias Cutáneas , Humanos , Sesgo , Neoplasias Cutáneas/prevención & control , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
Down syndrome (DS) has been related to a higher risk of hidradenitis suppurativa (HS). This cross-sectional study assessed DS patients with HS in a Spanish single-centre sample. DS participants presented a lower age of onset, age at diagnosis and time to diagnosis. Also, DS was not associated with the severity of HS measured by baseline IHS4 .
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Síndrome de Down , Hidradenitis Supurativa , Estudios Transversales , Síndrome de Down/complicaciones , Hidradenitis Supurativa/complicaciones , Hidradenitis Supurativa/diagnóstico , Hidradenitis Supurativa/epidemiología , Humanos , Índice de Severidad de la EnfermedadRESUMEN
BACKGROUND: Oral cavity cancer (OCC) and oropharyngeal cancer (OPC) are two common malignancies whose mortality is worryingly increasing worldwide. However, few studies have estimated the mortality trends for these cancers in the coming years. This study analysed the mortality rates for OCC and OPC observed between 1980 and 2019 to generate a predictive model for the next 25 years in Spain. METHODS: Mid-year population data and death certificates for the period 1980-2019 were obtained from the Spanish National Institute of Statistics. The Nordpred program (Norwegian Cancer Registry, Oslo, Norway) was used to calculate adjusted mortality rates as well as estimated mortality projections with an age-period-cohort model for the period 2020-2044. RESULTS: The specific mortality rate per 100,000 inhabitants for OCC decreased from 2.36 (1980-1984) to 2.17 (2015-2019) and is expected to decline to 1.68 (2040-2044), particularly in males. For OPC, mortality rates rose from 0.67 (1980-1984) to 1.23 (2015-2019) and are projected to drop to 0.71 (2040-2044). In the group of females > 65 years predictions showed rising mortality rates for both OCC and OPC. The predictive model projects more deaths in females than in males for OCC in the period 2040-2044, while deaths for OPC will decrease in males and gradually increase in females. CONCLUSIONS: Although OCC mortality rates have been found to decrease in males in the last observed decades, there is still room to improve them in females > 65 years in the future by promoting campaigns against smoking and alcohol consumption. OPC mortality will become a growing health problem. Vaccination campaigns for the prevention of human papillomavirus-associated cancers may have a long-term impact on the mortality of these cancers, which should be evaluated in upcoming studies. CLINICAL RELEVANCE: Our findings highlighted the importance of closely monitoring OCC and OPC mortality rates in the coming years by age group and sex, and the need to continue preventive measures against the main known risk factors, such as tobacco, alcohol, and human papillomavirus infection.
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Neoplasias de la Boca , Neoplasias Orofaríngeas , Femenino , Humanos , Incidencia , Masculino , Neoplasias de la Boca/patología , España/epidemiologíaRESUMEN
Malignant melanoma accounts for 80% of deaths due to skin cancer. Its incidence is globally increasing. However, melanoma mortality seems to be decreasing. The aim of this study was to analyze mortality rates due to melanoma in Andalusia between 1979 and 2018. Deaths due to melanoma and mid-year population in Andalusia were collected from the National Institute of Statistics. Age-adjusted mortality rates were calculated for overall population and for each sex and age group. Regression models were used to calculate significant points of change. Sex ratio and the independent effects of age, period, and cohort were also analyzed. Age-adjusted mortality due to melanoma rose from 0.61 to 1.94 deaths per 100.000 from 1979 to 2018 for the overall population. A significant change of trends was detected around 1994 when, after a steady rise from 1979, mortality rates stabilized up to the end of the period studied. The cited increase was more pronounced in >64 year males. From the end of the 2000s, there was a decrease in mortality rates to date in all population groups, producing a period effect. A stabilization in melanoma mortality rates was observed in Andalusia from 1994 with a decrease in some groups at the beginning of the 21st century. Trends observed in Andalusia do not differ substantially from those in Spain. The development of new therapies and an earlier diagnosis may have an influence in those changes. Studies that compare differences between Spanish regions are needed to define better prevention strategies.
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Melanoma , Neoplasias Cutáneas , Estudios de Cohortes , Humanos , Incidencia , Masculino , España/epidemiologíaRESUMEN
OBJECTIVE(S): To evaluate the frequency of nonmelanoma skin cancer (NMSC), NMSC precursors, and melanoma on a store-and-forward dermatology model featuring the pharmacist as the patient's point-of-contact. The secondary objective was to define lesion changes and symptoms perceived by patients (clinical prediction rules by nonexpert observers) that can be predictive of malignity. METHODS: A cross-sectional study of teledermatology consultation was performed. All patients who underwent a teledermatology consultation between September 2018 and March 2020 were included. A patient could have more than 1 lesion per consultation. The object of the study was a defined dermatologic lesion. The differences between the variables were analyzed using a univariate model based on the chi-square test for independent qualitative variables and Fisher exact test in cases when the expected values in any of the cells of a contingency table were less than 5. Statistical significance was set at P < 0.05 (2-tailed). RESULTS: A total of 225 lesions in 218 patients were considered for this study; 53.8% (n = 121) of the lesions were classified as benign, 16.4% (n = 37) as dubious, 23.1% (n = 52) as NMSC precursors, 5.8% (n = 13) as NMSC, and 0.9% (n = 2) as melanomas. Of the reported clinical lesion changes, spontaneous pain, pruritus, surface texture changes, color changes, or form changes had no statistically significant relationship with the diagnostic group, whereas the presence of spontaneous bleeding (P = 0.015) and size changes (P = 0.026) were more frequently observed in the "dubious lesion" and "of oncological relevance lesion" groups. CONCLUSION: This "direct-to-consumer," store-and-forward teledermatology with dermoscopy model featuring the pharmacist as the patient's point-of-contact is useful for the diagnosis of melanoma, NMSC, and NMSC precursors when backed by a robust dermatology service.
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Dermatología , Neoplasias Cutáneas , Telemedicina , Estudios Transversales , Dermoscopía , Humanos , Farmacéuticos , Neoplasias Cutáneas/diagnóstico por imagenRESUMEN
BACKGROUND: Recent studies suggest that Systemic lupus erythematosus (SLE) mortality rates in Spain are decreasing. However, SLE mortality in Spain has been poorly studied. The purpose of study is to assess the temporal trends of mortality rates in the Spanish population with SLE from 1980 to 2018. METHODS: Death records and mid-year population data were collected from the National Statistics Institute. Age-standardized mortality rates were calculated for overall population and for each sex and age group. Significant changes in mortality trends were identified by Joinpoint regressions. Also, an Age-period-cohort (APC) and Potential Years of Life Lost (PYLL) analysis was carried out to know the burden of SLE disease. RESULTS: The overall SLE mortality rates in Spain has experimented an increased through the last 39 years. Mortality rates from the period 1980-1984 was 0.83 per 1.000.000 inhabitants, reaching the value to 1.77 cases per 1.000.000 from the period 2014-2018. A decreasing trend has been observed since 1999. CONCLUSIONS: SLE mortality rate has increased in Spain between 1980 and 1999, with a sustained decrease up to our days.
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Lupus Eritematoso Sistémico/epidemiología , Adulto , Distribución por Edad , Anciano , Femenino , Humanos , Incidencia , Lupus Eritematoso Sistémico/mortalidad , Masculino , Persona de Mediana Edad , Análisis de Regresión , Distribución por Sexo , España/epidemiología , Tasa de Supervivencia/tendenciasRESUMEN
Chronic urticaria (CU) has a devastating effect on the quality of life of patients who suffer from it. The objective of this study is to determine the prevalence of severe chronic spontaneous urticaria (CSU) in adults in eight health areas in Andalucía. A retrospective observational study from January 2019 to December 2019 was conducted with the participation of seven reference hospitals that were analyzed, including a total reference population of 3 159 001 individuals ≥18 years. The analysis was carried out on the characteristics of the population and the estimated prevalence. In total, 369 patients with a diagnosis of CSU refractory to antagonist of histamine were registered. We have found a prevalence of refractory patients of 0.0012% (95% CI 0.0010%-0.0013), ranging from 0.006% to 0.02%. The present study has helped to describe the prevalence and characteristics of patients with severe urticarial. The prevalence is lower than previously reported. We have observed differences between hospital areas. There will be a new line of research at the national level to help improve the identification of patients with CSU.
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Urticaria Crónica , Urticaria , Adulto , Enfermedad Crónica , Antagonistas de los Receptores Histamínicos/uso terapéutico , Humanos , Omalizumab , Prevalencia , Calidad de Vida , España/epidemiología , Urticaria/diagnóstico , Urticaria/tratamiento farmacológico , Urticaria/epidemiologíaRESUMEN
Atopic dermatitis (AD) is a chronic, systemic disease that has a multifactorial etiology, where immune system disorders and epidermal barrier dysfunction are added to the influence of genetic and environmental factors. Dupilumab has been approved by United States and the European Union regulatory agencies in 2017 for the treatment of moderate-to-severe AD in adult patients who are candidates for systemic treatment. In this short report, we present a retrospective, multicentric study, in which five hospitals in Andalusia, Spain, have taken part. Our objective is to evaluate the safety and effectiveness of dupilumab in patients with moderate-to-severe AD treated with dupilumab with at least 52 weeks of follow-up.
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Anticuerpos Monoclonales Humanizados/administración & dosificación , Dermatitis Atópica/tratamiento farmacológico , Adulto , Anticuerpos Monoclonales Humanizados/efectos adversos , Dermatitis Atópica/patología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , España , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND AND OBJECTIVES: In recent years, the hypothesis that increasing the empowerment of patients can help to improve health outcomes and rationalize the use of health services has become relevant, especially for chronic diseases. Developing validated measurement tools is necessary to achieve this goal. In the field of dermatology, there are few studies related to empowerment of the patient. The aim of the present research is to develop and validate a self-administered questionnaire for adult patients diagnosed with atopic dermatitis (AD) in order to measure their level of empowerment. METHODS: After a literature review and the establishment of consulting groups of patients and dermatologists, a large battery of 35 items was generated, based on the dimensions included in previous empowerment descriptions. A multidisciplinary group selected the 20 most suitable items to include in the questionnaire. A cross-sectional study was carried out with the items included in the questionnaire as well as demographic and clinical characteristics. To reduce the number of items and measure the construct validity, an exploratory factorial analysis (EFA) of the primary components and varimax rotation were used. Cronbach's α was used to measure the reliability of the individual scales and the global questionnaire. RESULTS: 242 valid questionnaires were included. After completion of the EFA, the final Dermatitis Atópica EMPoderamiento (DATEMP) questionnaire was composed of 17 items that converged on four factors. The four scales had adequate reliability: "Knowledge" (Cronbach's alpha = 0.808), "Abilities" (Cronbach's alpha = 0.744), "Intention to change" (Cronbach's alpha = 0.798) and "Coping skills" (Cronbach's alpha = 0.772). The overall Cronbach's α of the questionnaire was 0.764. CONCLUSIONS: The DATEMP questionnaire is the first specific empowerment measurement instrument developed for patients with AD that has demonstrated adequate levels of reliability and construct validity. It is a self-administered questionnaire that is simple and quick to answer.
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Dermatitis Atópica/psicología , Empoderamiento , Encuestas y Cuestionarios/normas , Adaptación Psicológica , Adolescente , Adulto , Anciano , Estudios Transversales , Femenino , Conocimientos, Actitudes y Práctica en Salud , Humanos , Masculino , Persona de Mediana Edad , Proyectos Piloto , Tamaño de la Muestra , Adulto JovenRESUMEN
AIM: This study aims to elucidate the factors driving melanoma incidence trends in Spain by analyzing the GBD-2019 dataset (1990-2019) and investigating the age-specific, birth cohort, and period effects on incidence rates. MATERIALS AND METHODS: This study analyzed melanoma incidence trends in Spain from 1990 to 2019 using an ecological design. Data were sourced from the Global Burden of Disease Study 2019 and Spain's National Statistics Institute. Age-standardized incidence rates (ASIRs) were calculated using joinpoint regression analysis, and age-period-cohort (A-P-C) modeling was employed to assess the effects of age, time period, and birth cohort on incidence rates. RESULTS: Between 1990 and 2019, an estimated 147,823 melanoma cases were diagnosed in Spain. The ASIRs showed a steady increase for both sexes, with slightly higher rates observed in men. Both men (average annual percentage change (AAPC): 2.8%) and women (AAPC: 2.4%) showed a steady increase in the ASIR over the period. Joinpoint analysis revealed distinct periods of incidence rate changes, with significant upward trends in earlier years followed by stabilization in recent years. Incidence rates increased steadily with age, with the highest rates in the 80-84 age group. Women tended to have slightly higher rates in younger age groups, while men had higher rates in older age groups. Both men and women experienced a steady increase in relative risk of melanoma throughout the 30-year study period, with significant upward trends across birth cohorts. CONCLUSIONS: Despite limitations, this study provides valuable insights into factors influencing melanoma incidence in Spain. By understanding age, period, and cohort effects, effective prevention strategies can be developed to reduce melanoma incidence.
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Pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND) is a rare, monogenic, autoinflammatory disorder caused by mutations in exon 2 of the MEFV gene. Characterized by neutrophilic dermatosis, recurrent fever, and arthralgia, this syndrome presents a diagnostic challenge due to its low prevalence and varied clinical manifestations. Here, we present the case of a 49-year-old Spanish male with severe hidradenitis suppurativa and pyoderma gangrenosum with a heterozygous variant (p.E244K) in the MEFV gene, consistent with PAAND syndrome. This variant has only been documented in one other case with notable similarities. Both patients share Spanish ancestry and present a severe form of hidradenitis suppurativa. Treatment of the disorder presents challenges due to its variable response to standard therapies. Anti-interleukin-1 agents, such as anakinra or anti-tumor necrosis factor (TNF)-α are the therapeutic approaches supported by the most substantial evidence. Our findings highlight the importance of genetic evaluation of MEFV mutations in individuals with neutrophilic dermatosis and systemic symptoms.
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Background: Hand-foot eczema (HFE) are special locations of dermatitis, which are often associated with atopic dermatitis (AD) and have a significant negative impact on the quality of life, demanding a clinically relevant improvement. Objectives: To evaluate the effectiveness and safety of dupilumab in the treatment of eczema localized in hands and/or feet in patients with moderate-to-severe AD. Methods: Retrospective multicenter study of adult patients with HFE treated with dupilumab for their AD. Patients with other concomitantly systemic immunosuppressive treatments did not undergo a washout period. The severity of palmar and/or plantar involvement was assessed using the Physician Global Assessment (PGA) scale on a scale of 0 ( = clear) to 5 ( = very severe). Eczema Area and Severity Index (EASI) and NRS-pruritus scales were also evaluated. One hundred percent of patients reached week 16, while 67/84 reached week 52 of follow-up. Results: A total of 84 patients were included 86.69% of patients showed a reduction in PGA-Hand, and 80.34% in PGA-Foot at week 52, EASI improvement was reached by 83.55% of patients at week 16 and 87.35% at week 52. Reduction of pruritus (≥4 points in NRS-pruritus scale) was 73.01% at week 16 and 80.67% at week 52. No differences in response to dupilumab were observed in the different subtypes of palmo-plantar dermatitis. Conclusions: The results obtained in our study suggest that dupilumab may be an effective and safe therapeutic option for the treatment of dermatitis localized in hands and/or feet.