Assuntos
Dermatomiosite , Queimadura Solar , Dermatomiosite/diagnóstico , Hispânico ou Latino , Humanos , Pele , BronzeadoRESUMO
El síndrome de Osler-Weber-Rendu, o síndrome hereditario hemorrágico telangiectasia, es un trastorno raro de herencia autosómica dominante con una prevalencia estimada de 1:10.000 personas a nivel mundial. Las manifestaciones clínicas de este síndrome son resultado de malformaciones arteriovenosas y varían desde telangiectasias en piel y mucosas hasta afección de órganos sólidos que ponen en peligro la vida, como alteraciones hepáticas, émbolos sistémicos y fallo cardíaco, por lo cual el diagnóstico oportuno es de suma importancia para prevenir las complicaciones de la enfermedad y proporcionar apoyo genético a los familiares. En esta revisión se analiza el cuadro clínico con enfoque principal en las manifestaciones mucocutáneas de la enfermedad y su abordaje terapéutico
Osler-Weber-Rendu syndrome, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant disorder with an estimated worldwide prevalence of 1 case per 10,000 population. Its clinical manifestations are the result of arteriovenous malformations characterized by telangiectases that can affect the skin, mucous membranes, and solid organs and cause life-threatening conditions, such as liver disease, systemic emboli, and heart failure. Timely diagnosis is thus essential in order to prevent disease-related complications and offer genetic counseling to families. We review the clinical features of Osler-Weber-Rendu syndrome with a focus on mucocutaneous manifestations and their treatment
Assuntos
Humanos , Telangiectasia Hemorrágica Hereditária/epidemiologia , Dermatopatias/complicações , Telangiectasia Hemorrágica Hereditária/fisiopatologia , Telangiectasia/complicações , Epistaxe/complicaçõesRESUMO
Osler-Weber-Rendu syndrome, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant disorder with an estimated worldwide prevalence of 1 case per 10,000 population. Its clinical manifestations are the result of arteriovenous malformations characterized by telangiectases that can affect the skin, mucous membranes, and solid organs and cause life-threatening conditions, such as liver disease, systemic emboli, and heart failure. Timely diagnosis is thus essential in order to prevent disease-related complications and offer genetic counseling to families. We review the clinical features of Osler-Weber-Rendu syndrome with a focus on mucocutaneous manifestations and their treatment.
Assuntos
Telangiectasia Hemorrágica Hereditária/complicações , Malformações Arteriovenosas/complicações , Epistaxe/etiologia , Gastroenteropatias/etiologia , Humanos , Malformações Arteriovenosas Intracranianas/etiologia , Hepatopatias/etiologia , Pneumopatias/etiologia , Dermatopatias Vasculares/etiologia , Telangiectasia Hemorrágica Hereditária/diagnóstico , Telangiectasia Hemorrágica Hereditária/genética , Telangiectasia Hemorrágica Hereditária/terapiaAssuntos
Dermoscopia , Escabiose/diagnóstico por imagem , Adulto , Feminino , Humanos , Escabiose/patologiaAssuntos
Dermoscopia , Cisto Folicular/diagnóstico , Hamartoma/diagnóstico , Neoplasia de Células Basais/diagnóstico , Neoplasias Cutâneas/diagnóstico , Adulto , Diagnóstico Diferencial , Cisto Folicular/patologia , Hamartoma/patologia , Humanos , Masculino , Neoplasia de Células Basais/patologia , Neoplasias Cutâneas/patologiaRESUMO
Atypically presents in patients with pirexya, neutrophilia, painful tender erytomatous papules, nodules and plaques often distributed asymmetrically. Frequent sites include the face, neck and upper extremities. Affected sites show a characteristical neutrophilic infiltrate in the upper dermis. Its etiology remains elucidated, but it seems that can be mediated by a hypersensitivity reaction in which cytokines, followed by infiltration of neutrophils, may be involved. Systemic corticosteroids are the first-line of treatment in most cases. We present a concise review of the pathogenesis, classification, diagnosis and treatment update of this entity
El síndrome de Sweet es la entidad más representativa de las dermatosis neutrofílicas. Por lo general se presenta en pacientes con fiebre, neutrofilia, pápulas erytomatosas dolorosas, nódulos y placas. Los sitios frecuentemente afectados incluyen la cara, cuello y extremidades superiores los cuales característicamente presentan un infiltrado neutrofílico en la dermis superior. Su etiología no esta bien establecida, pero parece que puede estar mediada por una reacción de hipersensibilidad de las citocinas, seguido por un infiltrado de neutrófilos. Los corticosteroides sistémicos son la primera línea de tratamiento en la mayoría de los casos. Se presenta una revisión actual de la patogénesis, clasificación, diagnóstico y tratamiento de esta entidad
Assuntos
Humanos , Masculino , Feminino , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/etiologia , Síndrome de Sweet/patologia , Corticosteroides/administração & dosagem , Corticosteroides/farmacologia , Corticosteroides/uso terapêutico , Prevenção de Doenças , Diagnóstico , Recidiva , Seguimentos , Nível de Saúde , Terapêutica/instrumentação , Terapêutica/métodos , Neoplasias/etiologia , Neoplasias/patologia , Neoplasias/prevenção & controleRESUMO
Sweet syndrome is the most representative entity of febrile neutrophilic dermatoses. It typically presents in patients with pirexya, neutrophilia, painful tender erytomatous papules, nodules and plaques often distributed asymmetrically. Frequent sites include the face, neck and upper extremities. Affected sites show a characteristical neutrophilic infiltrate in the upper dermis. Its etiology remains elucidated, but it seems that can be mediated by a hypersensitivity reaction in which cytokines, followed by infiltration of neutrophils, may be involved. Systemic corticosteroids are the first-line of treatment in most cases. We present a concise review of the pathogenesis, classification, diagnosis and treatment update of this entity.
