RESUMO
Malignant atrophic papulosis sometimes known as Degos' disease is an idiopathic, uncommon condition with fewer than 200 occurrences documented. It is a chronic thrombo-obliterative vasculopathy characterised by papular skin lesions with a core porcelain-white atrophy and a surrounding telangiectatic border. We report a 15-year-old male patient with a recurrent history of hollow viscus perforation, which was managed on all the occasions with exploratory laparotomy and primary perforation repair. Additionally, the patient had a five month history of numerous, non-itchy, atrophic papules with a core porcelain-like area and hyperkeratotic margins, characteristic of Degos' disease. The only basis for diagnosis is the distinctive skin lesions with biopsy. Along with systemic lupus erythematosus and other connective tissue diseases, tuberculosis must also be taken into account while assessing the clinical presentation of malignant atrophic papulosis. There is currently no known treatment for malignant atrophic papulosis that has been effective. Keywords: case reports; intestinal perforation; malignant atrophic papulosis; ulcer; vasculitis.
Assuntos
Perfuração Intestinal , Papulose Atrófica Maligna , Dermatopatias , Masculino , Humanos , Adolescente , Papulose Atrófica Maligna/complicações , Papulose Atrófica Maligna/diagnóstico , Papulose Atrófica Maligna/tratamento farmacológico , Perfuração Intestinal/diagnóstico , Perfuração Intestinal/etiologia , Perfuração Intestinal/cirurgia , Porcelana Dentária/uso terapêutico , Diagnóstico Diferencial , Pele/patologiaRESUMO
The diagnosis of rabies, a potentially fatal neuroinfectious disease, should be strongly considered in all patients who develop encephalitis following an infected animal bite even when they have received post-exposure prophylaxis. In the absence of confirmatory tests, typical magnetic resonance imaging findings help confirm the clinical diagnosis of rabies.
