RESUMO
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.
Assuntos
Dermatologia , Venereologia , Humanos , Alopecia/tratamento farmacológico , Academias e Institutos , ConsensoRESUMO
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.
Assuntos
Malformações Arteriovenosas/patologia , Encéfalo/patologia , Capilares/anormalidades , Mancha Vinho do Porto/patologia , Pele/patologia , Coluna Vertebral/patologia , Malformações Vasculares/patologia , Adulto , Malformações Arteriovenosas/diagnóstico , Malformações Arteriovenosas/epidemiologia , Malformações Arteriovenosas/genética , Encéfalo/irrigação sanguínea , Capilares/patologia , Criança , Pré-Escolar , Análise de Dados , Feminino , Estudos de Associação Genética , Humanos , Achados Incidentais , Lactente , Masculino , Mutação , Mancha Vinho do Porto/diagnóstico , Mancha Vinho do Porto/epidemiologia , Mancha Vinho do Porto/genética , Prevalência , Receptor EphB4/genética , Pele/irrigação sanguínea , Espanha/epidemiologia , Coluna Vertebral/irrigação sanguínea , Malformações Vasculares/diagnóstico , Malformações Vasculares/genética , Proteína p120 Ativadora de GTPase/genéticaRESUMO
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.