RESUMEN
Androgenetic alopecia can be challenging to treat due to the wide range of available treatments, most of which are not based on evidence from clinical trials. In addition many of the options do not include androgenetic alopecia among the approved indications according to their summaries of product characteristics. A panel of 34 dermatologists from the Spanish Trichology Society of the Spanish Academy of Dermatology and Venereology (AEDV) used the Delphi method to develop a consensus statement on the management of androgenetic alopecia. Over a 2-round process the experts agreed on 138 (86%) of the 160 proposed items, which were structured into 4 blocks of recommendations: general considerations, pharmacologic treatment, procedures and hair transplant, and special cases. The resulting consensus statement based on expert opinion of the scientific evidence can guide professionals in the routine management of androgenetic alopecia.
Asunto(s)
Dermatología , Venereología , Humanos , Alopecia/tratamiento farmacológico , Academias e Institutos , ConsensoRESUMEN
BACKGROUND: Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is a rare syndrome with characteristic skin lesions that are associated with fast-flow vascular malformations (FFVMs) in one-third of patients. Few case series have been described, and none in Spain. AIM: To identify the prevalence of dermatological parameters, FFVMs and associated features in a large series of patients with CM-AVM. METHODS: We conducted an observational study of patients with CM-AVM syndrome diagnosed in 15 Spanish hospitals over 3 years. The main clinical, radiological, genetic findings and associated diseases were analysed. RESULTS: In total, 64 patients were assessed. In 26.5% of cases, the diagnosis was incidental. In 75% of patients, there was one significantly larger macule, which we termed the 'herald patch'. FFVMs were detected in 34% of the patients, with 30% located on the skin, 7.8% in the brain and in 1.5% in the spine. There was a positive family history in 65% of the 64 patients. Genetic analysis was performed for RASA1 mutations in 57 patients, of whom 42 (73%) had a positive result. All 4 patients tested for EPHB4 mutations had a positive result. No tumour lesions were detected in the series, except for five infantile haemangiomas. CONCLUSIONS: Our data on clinical lesions, associated FFVM, family history and genetics are similar to those previously published in the literature. An extensive data analysis failed to demonstrate any statistically significant association between the presence of an FFVM and any clinical, familial or genetic parameter that could predict its onset, although a link between the presence of a herald patch on the midline face and the presence of a brain FFVM was observed. We did not detect any genotype-phenotype correlation.
Asunto(s)
Malformaciones Arteriovenosas/patología , Encéfalo/patología , Capilares/anomalías , Mancha Vino de Oporto/patología , Piel/patología , Columna Vertebral/patología , Malformaciones Vasculares/patología , Adulto , Malformaciones Arteriovenosas/diagnóstico , Malformaciones Arteriovenosas/epidemiología , Malformaciones Arteriovenosas/genética , Encéfalo/irrigación sanguínea , Capilares/patología , Niño , Preescolar , Análisis de Datos , Femenino , Estudios de Asociación Genética , Humanos , Hallazgos Incidentales , Lactante , Masculino , Mutación , Mancha Vino de Oporto/diagnóstico , Mancha Vino de Oporto/epidemiología , Mancha Vino de Oporto/genética , Prevalencia , Receptor EphB4/genética , Piel/irrigación sanguínea , España/epidemiología , Columna Vertebral/irrigación sanguínea , Malformaciones Vasculares/diagnóstico , Malformaciones Vasculares/genética , Proteína Activadora de GTPasa p120/genéticaRESUMEN
IMPORTANCE: Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is a recently described syndrome with distinctive cutaneous lesions. Very little is known about the histopathology of these lesions. OBJECTIVE: The purpose of the study was to evaluate the histopathological characteristics of the pink macules of the CM-AVM syndrome and to investigate if these pink macules could be classified as capillary malformations or arteriovenous malformations based on their histopathological features. DESIGN-SETTINGS-PARTICIPANTS: We conducted a retrospective multicenter study involving eight hospitals in Spain. Fifteen biopsies from pink macules of the CM-AVM syndrome were analysed and compared with five biopsies of diverse capillary malformations and three stage I arteriovenous malformations. RESULTS: Pink macules' biopsies of the CM-AVM syndrome showed similar features including a high vascular density encompassing capillaries and numerous thick-walled arterioles mainly located in the superficial dermis, a predominance of elongated over round vessels, scarce or absent erythrocytes within the lumina and discrete perivascular inflammation. CMs were characterized by an increased number of capillary-type vessels mostly rounded and located in the upper dermis. AVMs were composed by highly increased numbers of vessels with a branching pattern involving the full thickness of the dermis, without erythrocytes within the lumina. Wilms tumour 1 protein was positive in the endothelial cells both in pink macules of the CM-AVM and in arteriovenous malformations. CONCLUSIONS AND RELEVANCE: Pink macules of the CM-AVM syndrome seem to be different from capillary malformations. Our results suggest that histologically and immunohistochemically they are closer to incipient arteriovenous malformations than to capillary malformations. A deepened knowledge about the nature of these skin lesions will contribute to the better understanding of capillary malformation-arteriovenous malformation syndrome, and will open the possibility of new and more specific treatments in the future.
Asunto(s)
Malformaciones Arteriovenosas , Capilares , Capilares/anomalías , Células Endoteliales , Humanos , Mancha Vino de Oporto , Estudios Retrospectivos , España , Proteína Activadora de GTPasa p120RESUMEN
El tratamiento de la alopecia androgénica (AGA) puede ser complejo para el clínico debido a la amplia gama de terapias disponibles, en muchos casos con escasos ensayos clínicos disponibles, y con muchas de las opciones de tratamiento sin aprobación de uso en la AGA según su ficha técnica. Este documento de consenso sobre el manejo de la AGA se ha elaborado siguiendo un método Delphi, en el que han participado 34 dermatólogos miembros del Grupo Español de Tricología de la Academia Española de Dermatología y Venereología. Tras 2 rondas de votaciones, se consensuaron 138 de los 160 ítems propuestos (86%), estructurados en 4 bloques de recomendaciones: generalidades, tratamiento farmacológico, procedimientos y trasplante capilar, y casos especiales. Este documento de consenso se apoya en la evidencia científica disponible y en la opinión de expertos para ayudar a los profesionales en el manejo de la AGA en la práctica clínica diaria.(AU)
Androgenetic alopecia can be challenging to treat due to the wide range of available treatments, most of which are not based on evidence from clinical trials. In addition many of the options do not include androgenetic alopecia among the approved indications according to their summaries of product characteristics. A panel of 34 dermatologists from the Spanish Trichology Society of the Spanish Academy of Dermatology and Venereology (AEDV) used the Delphi method to develop a consensus statement on the management of androgenetic alopecia. Over a 2-round process the experts agreed on 138 (86%) of the 160 proposed items, which were structured into 4 blocks of recommendations: general considerations, pharmacologic treatment, procedures and hair transplant, and special cases. The resulting consensus statement based on expert opinion of the scientific evidence can guide professionals in the routine management of androgenetic alopecia.(AU)
Asunto(s)
Humanos , Masculino , Femenino , Consenso , Terapias Complementarias , Alopecia/tratamiento farmacológico , Alopecia/terapia , España , DermatologíaRESUMEN
El tratamiento de la alopecia androgénica (AGA) puede ser complejo para el clínico debido a la amplia gama de terapias disponibles, en muchos casos con escasos ensayos clínicos disponibles, y con muchas de las opciones de tratamiento sin aprobación de uso en la AGA según su ficha técnica. Este documento de consenso sobre el manejo de la AGA se ha elaborado siguiendo un método Delphi, en el que han participado 34 dermatólogos miembros del Grupo Español de Tricología de la Academia Española de Dermatología y Venereología. Tras 2 rondas de votaciones, se consensuaron 138 de los 160 ítems propuestos (86%), estructurados en 4 bloques de recomendaciones: generalidades, tratamiento farmacológico, procedimientos y trasplante capilar, y casos especiales. Este documento de consenso se apoya en la evidencia científica disponible y en la opinión de expertos para ayudar a los profesionales en el manejo de la AGA en la práctica clínica diaria.(AU)
Androgenetic alopecia can be challenging to treat due to the wide range of available treatments, most of which are not based on evidence from clinical trials. In addition many of the options do not include androgenetic alopecia among the approved indications according to their summaries of product characteristics. A panel of 34 dermatologists from the Spanish Trichology Society of the Spanish Academy of Dermatology and Venereology (AEDV) used the Delphi method to develop a consensus statement on the management of androgenetic alopecia. Over a 2-round process the experts agreed on 138 (86%) of the 160 proposed items, which were structured into 4 blocks of recommendations: general considerations, pharmacologic treatment, procedures and hair transplant, and special cases. The resulting consensus statement based on expert opinion of the scientific evidence can guide professionals in the routine management of androgenetic alopecia.(AU)
Asunto(s)
Humanos , Masculino , Femenino , España , DermatologíaRESUMEN
La enfermedad de Darier segmentaria es una variante rara de esta patológica. Se caracteriza por una (..) (AU)
Segmental Darier disease is an unusual form of this condition. It is characterized by the localized (..) (AU)