Asunto(s)
Inmunosupresores/administración & dosificación , Metotrexato/administración & dosificación , Enfermedades de la Uña/dietoterapia , Psoriasis/tratamiento farmacológico , Adulto , Esquema de Medicación , Femenino , Estudios de Seguimiento , Humanos , Inmunosupresores/efectos adversos , Inyecciones Intralesiones , Masculino , Metotrexato/efectos adversos , Enfermedades de la Uña/diagnóstico , Enfermedades de la Uña/inmunología , Enfermedades de la Uña/patología , Uñas/efectos de los fármacos , Uñas/patología , Estudios Prospectivos , Psoriasis/diagnóstico , Psoriasis/inmunología , Psoriasis/patología , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Adulto JovenRESUMEN
A 10-year-old girl with yellow dystrophic nails, bronchiectasis, chronic sinusitis and lower-limb lymphedema is presented. The underlying mechanism remains unknown although it has been postulated to be associated with lymphatic abnormalities. To date no causative treatment exists. Our patient was treated with conservative management, including a low-fat diet supplemented with medium-chain triglycerides. Moderate improvement in the lymphedema of the lower extremities was observed. To our knowledge this is the first case of yellow nail syndrome to be treated with diet.
Asunto(s)
Bronquiectasia , Grasas de la Dieta/administración & dosificación , Linfedema/dietoterapia , Enfermedades de la Uña/dietoterapia , Sinusitis , Bronquiectasia/diagnóstico por imagen , Niño , Enfermedad Crónica , Femenino , Humanos , Síndrome , Tomografía Computarizada por Rayos XRESUMEN
A 26-year-old male patient with a history of chronic peripheral lymphedema, yellowish coloured slow growing nails and pleural effusions since early childhood is described. After 23 years he developed a chylous ascites and scintigraphy with technetium-99m labeled albumin clearly demonstrated a diffuse protein loss involving the whole jejunum and ileum. Subsequent jejunal and duodenal biopsies showed the typical histological findings of intestinal lymphangiectasia thereby confirming a diffuse intestinal lymphatic damage. In addition to the gastrointestional symptoms the patient developed a pericardial effusion diagnosed by echocardiographic imaging. Dietary treatment with middle chained triglycerides and intravenous human albumin supplementation was followed by the reduction of the ascites and improvement of the peripheral lymphedema. To our knowledge this is the first description of the yellow nail syndrome associated with a diffuse lymphangiectasia involving the whole small bowel.