RESUMO
Degos disease, also known as malignant atrophic papulosis, is a rare occlusive vasculopathy of unknown etiology characterized by infarcts in the dermis, gastrointestinal tract, central nervous system, and other organs. It is characterized by papules, which become umbilicated and evolve with a depressed porcelain-white central area, with an erythematous halo with telangiectasias. Histological findings include wedge-shaped dermoepidermal necrosis and blood vessel thrombosis. Approximately 50-60% of patients with systemic symptoms die within 2-3 years, most due to gastrointestinal perforation. We report a typical case, with lethal outcome, in a 45-year-old woman.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Pele/patologia , Papulose Atrófica Maligna/patologia , Biópsia , Evolução Fatal , Trombose Venosa/patologia , Papulose Atrófica Maligna/complicações , Perfuração Intestinal/complicaçõesAssuntos
Adulto , Feminino , Humanos , Isquemia Encefálica/etiologia , Papulose Atrófica Maligna/complicações , Aspirina/uso terapêutico , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/tratamento farmacológico , Fibrinolíticos/uso terapêutico , Papulose Atrófica Maligna/patologia , Papulose Atrófica Maligna/terapia , Ticlopidina/uso terapêuticoRESUMO
Degos disease DD is a rare obstructive vasculopathy characterized by distinctive skin lesions. Involvement of the soles, palms and genitalia is rare. In most cases, disease has an unfavorable course and involves gastrointestinal tract, central nervous system and occasionally other organs. Pleural and pericardial involvements are usually minor manifestations with prolonged course. Death occurs in approximately 50% of the patients usually due to intestinal perforation or central nervous system bleeding. We describe a 48-year-old man of lethal systemic DD. Widespread skin lesions with involvement of palmoplantar surfaces, genitalia and scalp were ignored for 3 years, whereas the disease revealed own malignant nature. The disorder progressed to nervous, gastrointestinal and cardiopulmonary system that led to death after 5 months from onset of systemic involvement as severe restrictive cardio-pulmonary insufficiency. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs