Sundaram Murugan, Thamotharan Nishantan, Swaminathan Adikrishnan, Rangarajan Sudha, Muralidhar Krishnakanth
Dermatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, IND.
Cureus. 2025 Jan 3;17(1):e76844. doi: 10.7759/cureus.76844. eCollection 2025 Jan.
Degos disease or malignant atrophic papulosis is a rare multisystem vascular disease of unknown etiology most commonly affecting the small- to medium-sized arteries of the dermis of the skin, gastrointestinal tract, and central nervous system. Endothelial cell damage and the activation of the coagulation cascade leading to the obliterative thrombosis of the vessel wall are the main pathogenic mechanisms behind the disease. The characteristic cutaneous features are porcelain white atrophic papules with a peripheral rim of erythema seen commonly over the trunk, chest, and upper and lower limbs. In Degos disease, cutaneous and systemic manifestations typically do not present simultaneously, with skin lesions often appearing first. Systemic involvement, affecting organs such as the gastrointestinal tract or central nervous system, may develop months or years later. Patients should be closely monitored for potential systemic complications, as these significantly impact prognosis, necessitating multidisciplinary care to ensure early detection and management. The disease carries a poor prognosis because of systemic involvement. Degos disease is extremely rare in children with very few cases being reported in literature. We hereby report a rare case of Degos disease in a five-year-old female child with lesions confined to the skin with a lack of systemic involvement.
迪戈斯病或恶性萎缩性丘疹病是一种病因不明的罕见多系统血管疾病，最常累及皮肤真皮层、胃肠道和中枢神经系统的中小动脉。内皮细胞损伤和凝血级联反应的激活导致血管壁闭塞性血栓形成是该疾病背后的主要致病机制。其特征性皮肤表现为瓷白色萎缩性丘疹，周围有红斑边缘，常见于躯干、胸部及上下肢。在迪戈斯病中，皮肤和全身表现通常不会同时出现，皮肤病变往往先出现。数月或数年后可能会出现影响胃肠道或中枢神经系统等器官的全身受累情况。应密切监测患者是否有潜在的全身并发症，因为这些并发症会显著影响预后，需要多学科护理以确保早期发现和处理。由于全身受累，该疾病预后较差。迪戈斯病在儿童中极为罕见，文献报道的病例很少。我们在此报告一例罕见的迪戈斯病，发生在一名5岁女童身上，病变局限于皮肤，无全身受累情况。
