Assuntos
Papulose Atrófica Maligna , Doenças do Pênis , Úlcera Cutânea , Humanos , Masculino , Doenças do Pênis/diagnóstico , Doenças do Pênis/patologia , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/patologia , Úlcera Cutânea/etiologia , Papulose Atrófica Maligna/diagnóstico , Papulose Atrófica Maligna/patologia , Papulose Atrófica Maligna/complicações , Pessoa de Meia-Idade , Dor/etiologia , Dor/diagnósticoRESUMO
A 49-year-old man presented with a 2-year history of weakness and sensory disturbances in the bilateral lower extremities, vesicorectal dysfunction, and progressive gait disturbances. Brain MRI revealed multiple ischemic and hemorrhagic cortical/subcortical lesions with patchy enhancement involving the frontal and parietal lobes, suggesting the possibility of distal perforating arteries injury. Spine MRI revealed lesions of the cervical and thoracic spinal cord with associated enhancement. The diagnosis of malignant atrophic papulosis (Degos disease) with central nervous system involvement was prompted by the characteristic skin lesions.
Assuntos
Papulose Atrófica Maligna , Dermatopatias , Masculino , Humanos , Pessoa de Meia-Idade , Papulose Atrófica Maligna/complicações , Papulose Atrófica Maligna/diagnóstico , Dermatopatias/complicações , Dermatopatias/patologia , Medula Espinal/patologiaRESUMO
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.