恶性萎缩性丘疹病的研究进展

Malignant atrophic papulosis

恶性萎缩性丘疹病是一种以萎缩性丘疹样皮损为特征的疾病，常伴有多系统脏器受累，死因多为胃肠穿孔和频发弥漫性腹膜炎。恶性萎缩性丘疹病发病原因尚不明，可能与免疫功能紊乱、高凝状态、纤维溶解蛋白异常、基因易感性、病毒和细菌感染有关。组织病理：血管腔纤维性增生和纤维蛋白血栓形成引起血管阻塞。免疫组化检查提示，膜攻击复合物、基质细胞衍生因子1／趋化因子12等因子有不同程度的沉积。恶性萎缩性丘疹病至今尚无有效的治疗方法，需要进一步研究。

Malignant atrophic papulosis is a rare disease characterized by atrophic papular skin lesions, and usually affects multiple systems and organs. Patients with malignant atrophic papulosis usually die from gastrointestinal perforation or frequent diffuse peritonitis. The exact pathogenesis of this disease is still unclear, and may be associated with immunological dysfunction, hypercoagulation, aberrant fibrinolysis, gene susceptibility, viral and bacterial infections. Histopathologically, it is characterized by vascular occlusion caused by fibrous hyperplasia and fibrinous thrombosis formation in lumina of blood vessels. As immunohistochemical staining shows, there are different degrees of deposites of C5b-9 and SDF-1/CXCL12. No effective treatment has been available for Degos disease up to now, and further researches are still needed.