Résumé
Malignant atrophic papulosisis is a rare, multisystem obliterative vasculopathy of unknown etiology, occasionally involving the cranial nerve. We describe the first case of malignant atrophic papulosisis with cranial nerve and peripheral nerve involvement in China. A 47-year-old woman presented to our hospital with atrophic porcelain white papules over the trunk and extremities, numbness in the right calf, vision decrease and impaired movement of the right eye. She was diagnosed with malignant atrophic papulosisis, based on characteristic symptoms and histopathologic examination. The patient was treated with dipyridamole and aspirin for 9 months, but later died of gastrointestinal hemorrhage. We reviewed currently available case reports on cranial nerve involvement in malignant atrophic papulosisis and emphasized the importance of skin biopsy in diagnosing this disease.
.Sujets)
Femelle , Humains , Adulte d'âge moyen , Atteintes des nerfs crâniens/anatomopathologie , Papulose atrophiante maligne/anatomopathologie , Neuropathies périphériques/anatomopathologie , Biopsie , Atteintes des nerfs crâniens/traitement médicamenteux , Issue fatale , Papulose atrophiante maligne/traitement médicamenteux , Neuropathies périphériques/traitement médicamenteux , Peau/anatomopathologieRésumé
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.
Sujets)
Humains , Femelle , Adulte d'âge moyen , Peau/anatomopathologie , Papulose atrophiante maligne/anatomopathologie , Biopsie , Issue fatale , Thrombose veineuse/anatomopathologie , Papulose atrophiante maligne/complications , Perforation intestinale/complicationsRésumé
Presentamos una paciente de sexo femenino, de 53 años de edad, con manifestaciones cutáneas de enfermedad de Degos, sin compromiso sistémico asociado. Se realiza una revisión actualizada de la bibliografía médica mundial, con una descripción clínica, histopatológica, diagnóstica y terapéutica de la enfermedad.