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Han Dawei, Wang Tao, Liu Yuehua. Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China Corresponding author: Liu Yuehua, Email: yuehualiu@263.net ��Abstract�� 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.