恶性萎缩性丘疹病又称为Degos病，是一种罕见的不明原因的血管性疾病，主要累及皮肤、胃肠道和中枢神经系统。 Degos病的临床表现为许多直径0 G．5～1 cm的中心瓷白色丘疹。其发病机制尚不完全清楚，近年研究发现SDF-1／CXCL12、C5b-9参与了恶性萎缩性丘疹病的发病，一些新的治疗方法如Eculizumab、曲前列尼尔、糖皮质激素联合静脉注射免疫球蛋白也取得了一定疗效。文章对恶性萎缩性丘疹病的病因，发病机制，诊断和治疗等进展进行综述。“,”Malignant atrophic papulosis , also known as Degos disease ,is a rare vasculopathy of unknown cause that mainly involves the skin , gastrointestinal tract , central nervous system .The clinicopathologic finding of Degos disease is many papules,0.5-1cm in diameter, with the classic porcelain-white centers.The pathogenesis of Degos disease is not completely understood.Some studies have found that SDF-1/CXCL12 and C5b-9 may be involved in the pathogenesis of the disease in re-cent years .Some new therapies such as eculizumab、treprostinil and corticosteroid combined with intravenous immunoglobulin have got certain therapeutic efficacy .The paper reviewed the progress of malignant atrophic papulosis on etiology , pathogene-sis, diagnosis, treatment ,and so on.