Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 192
Filtrar
2.
Actas dermo-sifiliogr. (Ed. impr.) ; 114(5): 382-391, mayo 2023. ilus, tab, graf
Artigo em Inglês | IBECS | ID: ibc-220776

RESUMO

Background and objective Real-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines. Material and method This retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020. Results Survey data from 57 treating physicians were included (71.9% [N=41] dermatologists, 17.6% [N=10] general practitioners/primary care physicians, and 10.5% [N=6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively. Conclusions These real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines (AU)


Antecedente y objetivo En España se carece de evidencia sobre psoriasis (PsO) pediátrica en el mundo real. El objetivo de este estudio fue identificar la carga de la enfermedad reportada por el facultativo y los patrones actuales de tratamiento en una cohorte de pacientes psoriásicos pediátricos en el mundo real. Ello ampliará nuestra comprensión de la enfermedad y contribuirá al desarrollo de directrices regionales. Material y método Este análisis retrospectivo transversal de una encuesta de investigación mercado evaluó las necesidades clínicas no satisfechas y los patrones de tratamiento en pacientes con PsO pediátrica en España, según lo reportado por sus médicos de atención primaria y especialistas, utilizando datos recopilados como parte del Disease-specific program (DSP™) de Adelphi para PsO pediátrica en el mundo real, entre los meses de febrero y octubre de 2020. Resultados Se incluyeron los datos de la encuesta realizada a 57 facultativos médicos tratantes (71,9% [N=41] de dermatólogos, 17,6% [N=10] de médicos generales de atención primaria y 10,5% [N=6] de pediatras); el análisis final incluyó 378 pacientes. En la muestra, el 84,1% (318/378) de los pacientes padeció enfermedad leve, el 15,3% (58/378) enfermedad moderada y el 0,5% (2/378) enfermedad severa. De acuerdo con el reporte retrospectivo, la gravedad juzgada por el facultativo en el momento de diagnosticarse la PsO pediátrica registró un 41,8% (158/378) de pacientes con enfermedad leve, un 51,3% (194/378) con enfermedad moderada y un 6,9% (26/378) con enfermedad severa. En general, el 89,3% (335/375) de los pacientes recibía en la actualidad terapia tópica para PsO pediátrica, mientras que el 8,8% (33/375), el 10,4% (39/375) y el 14,9% (56/375) de los pacientes recibía en la actualidad fototerapia, sistémicos y biológicos convencionales, respectivamente. Conclusiones Estos datos del mundo real reflejan la carga actual y el panorama de la PsO pediátrica en España (AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Pesquisas sobre Atenção à Saúde , Efeitos Psicossociais da Doença , Psoríase/terapia , Índice de Gravidade de Doença , Estudos Transversais , Estudos Retrospectivos , Espanha
3.
Actas dermo-sifiliogr. (Ed. impr.) ; 114(5): t382-t381, mayo 2023. ilus, tab, graf
Artigo em Espanhol | IBECS | ID: ibc-220777

RESUMO

Antecedente y objetivo En España se carece de evidencia sobre psoriasis (PsO) pediátrica en el mundo real. El objetivo de este estudio fue identificar la carga de la enfermedad reportada por el facultativo y los patrones actuales de tratamiento en una cohorte de pacientes psoriásicos pediátricos en el mundo real. Ello ampliará nuestra comprensión de la enfermedad y contribuirá al desarrollo de directrices regionales. Material y método Este análisis retrospectivo transversal de una encuesta de investigación mercado evaluó las necesidades clínicas no satisfechas y los patrones de tratamiento en pacientes con PsO pediátrica en España, según lo reportado por sus médicos de atención primaria y especialistas, utilizando datos recopilados como parte del Disease-specific program (DSP™) de Adelphi para PsO pediátrica en el mundo real, entre los meses de febrero y octubre de 2020. Resultados Se incluyeron los datos de la encuesta realizada a 57 facultativos médicos tratantes (71,9% [N=41] de dermatólogos, 17,6% [N=10] de médicos generales de atención primaria y 10,5% [N=6] de pediatras); el análisis final incluyó 378 pacientes. En la muestra, el 84,1% (318/378) de los pacientes padeció enfermedad leve, el 15,3% (58/378) enfermedad moderada y el 0,5% (2/378) enfermedad severa. De acuerdo con el reporte retrospectivo, la gravedad juzgada por el facultativo en el momento de diagnosticarse la PsO pediátrica registró un 41,8% (158/378) de pacientes con enfermedad leve, un 51,3% (194/378) con enfermedad moderada y un 6,9% (26/378) con enfermedad severa. En general, el 89,3% (335/375) de los pacientes recibía en la actualidad terapia tópica para PsO pediátrica, mientras que el 8,8% (33/375), el 10,4% (39/375) y el 14,9% (56/375) de los pacientes recibía en la actualidad fototerapia, sistémicos y biológicos convencionales, respectivamente. Conclusiones Estos datos del mundo real reflejan la carga actual y el panorama de la PsO pediátrica en España (AU)


Background and objective Real-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines. Material and method This retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020. Results Survey data from 57 treating physicians were included (71.9% [N=41] dermatologists, 17.6% [N=10] general practitioners/primary care physicians, and 10.5% [N=6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively. Conclusions These real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines (AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Pesquisas sobre Atenção à Saúde , Efeitos Psicossociais da Doença , Psoríase/terapia , Índice de Gravidade de Doença , Estudos Transversais , Estudos Retrospectivos , Espanha
4.
Actas Dermosifiliogr ; 114(5): T382-T391, 2023 May.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-37088286

RESUMO

BACKGROUND AND OBJECTIVE: Real-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines. MATERIAL AND METHOD: This retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020. RESULTS: Survey data from 57 treating physicians were included (71.9% [N = 41] dermatologists, 17.6% [N = 10] general practitioners/primary care physicians, and 10.5% [N = 6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively. CONCLUSIONS: These real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines.


Assuntos
Psoríase , Humanos , Criança , Estudos Retrospectivos , Espanha/epidemiologia , Estudos Transversais , Psoríase/terapia , Psoríase/tratamento farmacológico , Fototerapia
5.
Actas Dermosifiliogr ; 114(5): 382-391, 2023 May.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-36907550

RESUMO

BACKGROUND AND OBJECTIVE: Real-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines. MATERIAL AND METHOD: This retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020. RESULTS: Survey data from 57 treating physicians were included (71.9% [N=41] dermatologists, 17.6% [N=10] general practitioners/primary care physicians, and 10.5% [N=6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively. CONCLUSIONS: These real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines.


Assuntos
Psoríase , Humanos , Criança , Estudos Retrospectivos , Espanha/epidemiologia , Estudos Transversais , Psoríase/terapia , Psoríase/tratamento farmacológico , Fototerapia
7.
Actas dermo-sifiliogr. (Ed. impr.) ; 113(10): 923-929, nov.- dic. 2022. ilus, tab, graf
Artigo em Inglês | IBECS | ID: ibc-213026

RESUMO

Background The diagnosis of Neurofibromatosis type 1 (NF1) is usually delayed in children without a family history. We aimed to define the prevalence and characteristics of prevalent skin manifestations in NF1 compared to the general population, which continue to be excluded from the diagnostic criteria for NF1. Patients and methods Case–control study, matched by age groups, in which 108 patients with a diagnosis of NF1 and 137 healthy controls were included. Results The prevalence of nevus anemicus (NA) (p<0.001) and juvenile xanthogranulomas (JXG) (p<0.001) was significantly higher in the population affected by NF1 than in the control population. A specificity of 99.27% [confidence interval (CI): 95.4–99.96%] and a positive predictive value (PPV) of 98.80% [92.54–99.94%] were estimated for NA and a specificity of 99.27% [95.4–99.96%] and a PPV of 92.86% [64.17–99.63%] for JXG in the diagnosis of NF1 in children who present 6 or more Café-au-lait macules. Statistically significant differences were also evidenced in the distribution by phototypes (p 0.025) and in relation to generalized itching with no other cause (p<0.001). Conclusions NA and JXG are relevant clinical findings for the diagnosis of NF1, especially during the first years of life. We consider that its inclusion among the diagnostic criteria of the disease should be evaluated (AU)


Antecedentes El diagnóstico de la neurofibromatosis tipo 1 (NF1) se demora normalmente en niños sin antecedentes familiares. Nuestro objetivo fue definir la prevalencia y características de las manifestaciones cutáneas prevalentes en NF1, en comparación con la población general, que siguen siendo excluidas de los criterios diagnósticos para NF1. Pacientes y métodos Estudio de casos y controles, pareado por grupos de edad, en el que se incluyeron 108 pacientes diagnosticados de NF1 y 137 controles sanos. Resultados La prevalencia de nevus anemicus (NA) (p < 0,001) y xantogranuloma juvenil (XJ) (p < 0,001) fue significativamente superior en la población afectada de NF1, en comparación con el grupo control. Se estimaron una especificidad del 99,27% [Intervalo de confianza (IC): 95,4-99,96%] y un valor predictivo positivo (VPP) del 98,80% [92,54-99,94%] para NA, y una especificidad del 99,27% [95,4-99,96%] y VPP del 92,86% [64,17-99,63%] para XJ en el diagnóstico de NF1 en niños que presentan 6 o más manchas café con leche. También se evidenciaron diferencias estadísticamente significativas en la distribución por fototipos (p 0,025), y con relación al prurito generalizado sin ninguna otra causa (p <,001). Conclusiones Los NA y los XJ son hallazgos clínicos relevantes para el diagnóstico de NF1, especialmente durante los primeros años de vida. Consideramos que debería evaluarse su inclusión en los criterios diagnósticos de la enfermedad (AU)


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Manchas Café com Leite/diagnóstico , Neurofibromatose 1/diagnóstico , Transtornos da Pigmentação/diagnóstico , Xantogranuloma Juvenil/diagnóstico , Estudos de Casos e Controles , Estudos Transversais
8.
Actas dermo-sifiliogr. (Ed. impr.) ; 113(10): t923-t929, nov.- dic. 2022. ilus, tab, graf
Artigo em Espanhol | IBECS | ID: ibc-213027

RESUMO

Antecedentes El diagnóstico de la neurofibromatosis tipo 1 (NF1) se demora normalmente en niños sin antecedentes familiares. Nuestro objetivo fue definir la prevalencia y características de las manifestaciones cutáneas prevalentes en NF1, en comparación con la población general, que siguen siendo excluidas de los criterios diagnósticos para NF1. Pacientes y métodos Estudio de casos y controles, pareado por grupos de edad, en el que se incluyeron 108 pacientes diagnosticados de NF1 y 137 controles sanos. Resultados La prevalencia de nevus anemicus (NA) (p < 0,001) y xantogranuloma juvenil (XJ) (p < 0,001) fue significativamente superior en la población afectada de NF1, en comparación con el grupo control. Se estimaron una especificidad del 99,27% [Intervalo de confianza (IC): 95,4-99,96%] y un valor predictivo positivo (VPP) del 98,80% [92,54-99,94%] para NA, y una especificidad del 99,27% [95,4-99,96%] y VPP del 92,86% [64,17-99,63%] para XJ en el diagnóstico de NF1 en niños que presentan 6 o más manchas café con leche. También se evidenciaron diferencias estadísticamente significativas en la distribución por fototipos (p 0,025), y con relación al prurito generalizado sin ninguna otra causa (p <,001). Conclusiones Los NA y los XJ son hallazgos clínicos relevantes para el diagnóstico de NF1, especialmente durante los primeros años de vida. Consideramos que debería evaluarse su inclusión en los criterios diagnósticos de la enfermedad (AU)


Background The diagnosis of Neurofibromatosis type 1 (NF1) is usually delayed in children without a family history. We aimed to define the prevalence and characteristics of prevalent skin manifestations in NF1 compared to the general population, which continue to be excluded from the diagnostic criteria for NF1. Patients and methods Case–control study, matched by age groups, in which 108 patients with a diagnosis of NF1 and 137 healthy controls were included. Results The prevalence of nevus anemicus (NA) (p<0.001) and juvenile xanthogranulomas (JXG) (p<0.001) was significantly higher in the population affected by NF1 than in the control population. A specificity of 99.27% [confidence interval (CI): 95.4–99.96%] and a positive predictive value (PPV) of 98.80% [92.54–99.94%] were estimated for NA and a specificity of 99.27% [95.4–99.96%] and a PPV of 92.86% [64.17–99.63%] for JXG in the diagnosis of NF1 in children who present 6 or more Café-au-lait macules. Statistically significant differences were also evidenced in the distribution by phototypes (p 0.025) and in relation to generalized itching with no other cause (p<0.001). Conclusions NA and JXG are relevant clinical findings for the diagnosis of NF1, especially during the first years of life. We consider that its inclusion among the diagnostic criteria of the disease should be evaluated (AU)


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Manchas Café com Leite/diagnóstico , Neurofibromatose 1/diagnóstico , Transtornos da Pigmentação/diagnóstico , Xantogranuloma Juvenil/diagnóstico , Estudos de Casos e Controles , Estudos Transversais
9.
Actas Dermosifiliogr ; 113(10): T923-T929, 2022.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-36162491

RESUMO

BACKGROUND: The diagnosis of Neurofibromatosis type 1 (NF1) is usually delayed in children without a family history. We aimed to define the prevalence and characteristics of prevalent skin manifestations in NF1 compared to the general population, which continue to be excluded from the diagnostic criteria for NF1. PATIENTS AND METHODS: Case-control study, matched by age groups, in which 108 patients with a diagnosis of NF1 and 137 healthy controls were included. RESULTS: The prevalence of nevus anemicus (NA) (P<.001) and juvenile xanthogranulomas (JXG) (P<.001) was significantly higher in the population affected by NF1 than in the control population. A specificity of 99.27% (confidence interval): 95.4-99.96%] and a positive predictive value (PPV) of 98.80% [92.54-99.94%] were estimated for NA and a specificity of 99.27% [95.4-99.96%] and a PPV of 92.86% [64.17-99.63%] for JXG in the diagnosis of NF1 in children who present 6 or more Café-au-lait macules. Statistically significant differences were also evidenced in the distribution by phototypes (P=.025) and in relation to generalized itching with no other cause (P<.001). CONCLUSIONS: NA and JXG are relevant clinical findings for the diagnosis of NF1, especially during the first years of life. We consider that its inclusion among the diagnostic criteria of the disease should be evaluated.


Assuntos
Neurofibromatose 1 , Transtornos da Pigmentação , Xantogranuloma Juvenil , Criança , Humanos , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/epidemiologia , Estudos de Casos e Controles , Manchas Café com Leite/diagnóstico , Prevalência , Inflamação
10.
J Eur Acad Dermatol Venereol ; 36(11): 1904-1926, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36056736

RESUMO

The evidence- and consensus-based guideline on atopic eczema was developed in accordance with the EuroGuiDerm Guideline and Consensus Statement Development Manual. Four consensus conferences were held between December 2020 and July 2021. Twenty-nine experts (including clinicians and patient representatives) from 12 European countries participated. This second part of the guideline includes recommendations and detailed information on basic therapy with emollients and moisturizers, topical anti-inflammatory treatment, antimicrobial and antipruritic treatment and UV phototherapy. Furthermore, this part of the guideline covers techniques for avoiding provocation factors, as well as dietary interventions, immunotherapy, complementary medicine and educational interventions for patients with atopic eczema and deals with occupational and psychodermatological aspects of the disease. It also contains guidance on treatment for paediatric and adolescent patients and pregnant or breastfeeding women, as well as considerations for patients who want to have a child. A chapter on the patient perspective is also provided. The first part of the guideline, published separately, contains recommendations and guidance on systemic treatment with conventional immunosuppressive drugs, biologics and janus kinase (JAK) inhibitors, as well as information on the scope and purpose of the guideline, and a section on guideline methodology.


Assuntos
Anti-Infecciosos , Produtos Biológicos , Dermatite Atópica , Fármacos Dermatológicos , Eczema , Adolescente , Anti-Infecciosos/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Antipruriginosos/uso terapêutico , Produtos Biológicos/uso terapêutico , Criança , Dermatite Atópica/tratamento farmacológico , Fármacos Dermatológicos/uso terapêutico , Eczema/tratamento farmacológico , Emolientes/uso terapêutico , Feminino , Humanos , Janus Quinases
11.
J Eur Acad Dermatol Venereol ; 36(9): 1409-1431, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35980214

RESUMO

The evidence- and consensus-based guideline on atopic eczema was developed in accordance with the EuroGuiDerm Guideline and Consensus Statement Development Manual. Four consensus conferences were held between December 2020 and July 2021. Twenty-nine experts (including clinicians and patient representatives) from 12 European countries participated. This first part of the guideline includes general information on its scope and purpose, the health questions covered, target users and a methods section. It also provides guidance on which patients should be treated with systemic therapies, as well as recommendations and detailed information on each systemic drug. The systemic treatment options discussed in the guideline comprise conventional immunosuppressive drugs (azathioprine, ciclosporin, glucocorticosteroids, methotrexate and mycophenolate mofetil), biologics (dupilumab, lebrikizumab, nemolizumab, omalizumab and tralokinumab) and janus kinase inhibitors (abrocitinib, baricitinib and upadacitinib). Part two of the guideline will address avoidance of provocation factors, dietary interventions, immunotherapy, complementary medicine, educational interventions, occupational and psychodermatological aspects, patient perspective and considerations for paediatric, adolescent, pregnant and breastfeeding patients.


Assuntos
Dermatite Atópica , Eczema , Adolescente , Azatioprina/uso terapêutico , Criança , Ciclosporina/uso terapêutico , Dermatite Atópica/tratamento farmacológico , Eczema/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , Ácido Micofenólico/uso terapêutico
13.
Actas Dermosifiliogr ; 113(10): 923-929, 2022.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-35636506

RESUMO

BACKGROUND: The diagnosis of Neurofibromatosis type 1 (NF1) is usually delayed in children without a family history. We aimed to define the prevalence and characteristics of prevalent skin manifestations in NF1 compared to the general population, which continue to be excluded from the diagnostic criteria for NF1. PATIENTS AND METHODS: Case-control study, matched by age groups, in which 108 patients with a diagnosis of NF1 and 137 healthy controls were included. RESULTS: The prevalence of nevus anemicus (NA) (p<0.001) and juvenile xanthogranulomas (JXG) (p<0.001) was significantly higher in the population affected by NF1 than in the control population. A specificity of 99.27% [confidence interval (CI): 95.4-99.96%] and a positive predictive value (PPV) of 98.80% [92.54-99.94%] were estimated for NA and a specificity of 99.27% [95.4-99.96%] and a PPV of 92.86% [64.17-99.63%] for JXG in the diagnosis of NF1 in children who present 6 or more Café-au-lait macules. Statistically significant differences were also evidenced in the distribution by phototypes (p 0.025) and in relation to generalized itching with no other cause (p<0.001). CONCLUSIONS: NA and JXG are relevant clinical findings for the diagnosis of NF1, especially during the first years of life. We consider that its inclusion among the diagnostic criteria of the disease should be evaluated.


Assuntos
Neurofibromatose 1 , Transtornos da Pigmentação , Xantogranuloma Juvenil , Criança , Humanos , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/epidemiologia , Estudos de Casos e Controles , Manchas Café com Leite/epidemiologia , Manchas Café com Leite/etiologia , Manchas Café com Leite/diagnóstico , Prevalência , Inflamação
17.
J Eur Acad Dermatol Venereol ; 35(10): 2079-2084, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33988879

RESUMO

BACKGROUND: Maculopapular cutaneous mastocytosis (MPCM) in children is classified in two variants: (i) monomorphic variant, presenting with the small macules or papules typically seen in adult patients; and (ii) polymorphic variant with larger lesions of variable size and shape, typically seen in children. The definition of polymorphic and monomorphic variants is mostly intuitive, and a validation of this classification has not been done. OBJECTIVE: To study interobserver variability in the classification of MPCM in two groups of observers: mastocytosis experts and general dermatologists. MATERIALS AND METHODS: Nineteen cases of childhood MPCM were shown blindly, for classification as monomorphic or polymorphic type, to 10 independent observers (eight dermatologists, one allergist and one haematologist) from Europe and North America with a vast experience in the management of paediatric mastocytosis. Also, the same cases were shown on a screen to 129 general dermatologists attending a meeting; their votes were registered by remote controls. The interobserver variability kappa coefficient (with 95% confidence interval) was calculated to measure the reliability of the correlation. RESULTS: The value of kappa interobserver variability coefficient for the group of 10 experts (95% confidence interval) was 0.39 (0.18-0.63), which is considered as 'fair'. The value of kappa interobserver variability coefficient for the group of 129 general dermatologists (95% confidence interval) was 0.17 (0.06-0.39), which is considered as 'slight'. A complete agreement of all 10 experts was achieved in only four of 19 cases (21.1%) The most voted choice was concordant between the two groups in only 11 of the 19 cases. CONCLUSIONS: We failed to validate the classification system of childhood MPCM in monomorphic and polymorphic types. While the rate of agreement was low for mastocytosis experts, it was nearly the agreement expected by chance in general dermatologists.


Assuntos
Urticaria Pigmentosa , Adulto , Criança , Europa (Continente) , Humanos , Variações Dependentes do Observador , Reprodutibilidade dos Testes
20.
Clin Exp Dermatol ; 46(3): 444-450, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33180982

RESUMO

The current COVID-19 pandemic is caused by the SARS-CoV-2 coronavirus. The initial recognized symptoms were respiratory, sometimes culminating in severe respiratory distress requiring ventilation, and causing death in a percentage of those infected. As time has passed, other symptoms have been recognized. The initial reports of cutaneous manifestations were from Italian dermatologists, probably because Italy was the first European country to be heavily affected by the pandemic. The overall clinical presentation, course and outcome of SARS-CoV-2 infection in children differ from those in adults as do the cutaneous manifestations of childhood. In this review, we summarize the current knowledge on the cutaneous manifestations of COVID-19 in children after thorough and critical review of articles published in the literature and from the personal experience of a large panel of paediatric dermatologists in Europe. In Part 1, we discuss one of the first and most widespread cutaneous manifestation of COVID-19, chilblain-like lesions. In Part 2, we review other manifestations, including erythema multiforme, urticaria and Kawasaki disease-like inflammatory multisystemic syndrome, while in Part 3, we discuss the histological findings of COVID-19 manifestations, and the testing and management of infected children, for both COVID-19 and any other pre-existing conditions.


Assuntos
COVID-19/complicações , Pérnio/virologia , Adolescente , COVID-19/diagnóstico , COVID-19/patologia , COVID-19/terapia , Teste para COVID-19 , Pérnio/imunologia , Pérnio/patologia , Criança , Humanos , Interferon Tipo I/imunologia , Remissão Espontânea , Fatores de Risco , SARS-CoV-2 , Trombose/etiologia , Vasculite/etiologia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...