RESUMEN
Las ictiosis hereditarias son un grupo de trastornos genéticos de la cornificación, que se caracterizan por presentar hiperqueratosis y/o descamación. La nueva clasificación identifica 36 tipos de ictiosis, las cuales se subdividen según su frecuencia, patrón de herencia y compromiso extracutáneo. El diagnóstico se basa principalmente en las características clínicas, ya que los estudios genéticos no se encuentran disponibles en nuestro medio. El tratamiento es sintomático y su manejo debe ser realizado por un equipo multidisciplinario. En este artículo se revisan los aspectos diagnósticos y terapéuticos de los distintos tipos de ictiosis, considerando la nomenclatura y modificaciones expuestas en la nueva clasificación.
Hereditary ichthyoses are a group of genetic disorders of cornification, which are characterised by hyperkeratosis and scaling. The new classification identifies 36 types of ichthyosis, which are subdivided according to their frequency, pattern of inheritance and extracutaneous involvement. The diagnosis is mainly based on clinical features, since genetic studies are not available in our setting. Treatment is symptomatic and management should be performed by a multidisciplinary team. In this article, the diagnostic and therapeutic aspects of different types of ichthyosis are reviewed, taking into account the nomenclature and modifications presented in the new classification.
Asunto(s)
Humanos , Grupo de Atención al Paciente/organización & administración , Ictiosis/genética , Ictiosis/diagnóstico , Ictiosis/terapia , Terminología como AsuntoRESUMEN
Background: Pyoderma gangrenosum is an ulcerative disease of the skin of unknown cause and generally associated to systemic illnesses. It requires an aggressive systemic therapy and there is little information about its long term evolution. Aim: To report the clinical features of patients with pyoderma gangrenosum. Patients and methods: Eleven patients (10 female) aged 27 to 81 years old with pyoderma gangrenosum are reported. All had a pathological study. Patients were followed up for a mean of 60 months. Results: The lesions were located in the lower limbs in 8 patients. Pathological study showed neutrophyl infiltrates in nine patients. Three patients had an ulcerative colitis and one, a Crohn disease. During the follow up, the disease recurred in multiple occasions, in six patients, after treatment with steroids or sulphones, and one patient has had permanently active lesions. Conclusions: Pyoderma gangrenosum appears mostly in the lower limbs and has a high recurrence rate