Ojeda Cuchillero R M, Sánchez Regaña M, Umbert Millet P
Department of Dermatology, Hospital Sagrado Corazón, Teaching Unit of University of Barcelona, Barcelona, Spain.
Clin Exp Dermatol. 2003 Mar;28(2):145-7. doi: 10.1046/j.1365-2230.2003.01161.x.
Malignant atrophic papulosis is a rare systemic vaso-occlusive disorder characterized by thrombosis of vessels of the dermis, gastrointestinal tract, central nervous system and, occasionally, other organs. Cutaneous lesions consist of erythematous, dome-shaped papules that develop a central area of necrosis to leave a porcelain-like scar. The most accepted theory of pathogenesis is based on endothelial cell damage. There is no effective treatment of the disease. We describe a 26-year-old man with Degos' disease, a diagnosis based on the clinical and histologic pattern of skin lesions. The good response to antiplatelet therapy and the absence of systemic involvement over 8 years' follow-up is noteworthy. We believe that this case represents the benign form of the disease, typically referred to as benign cutaneous Degos' disease.
恶性萎缩性丘疹病是一种罕见的系统性血管闭塞性疾病，其特征为真皮、胃肠道、中枢神经系统以及偶尔其他器官的血管发生血栓形成。皮肤损害由红斑性、圆顶状丘疹组成，这些丘疹会形成中央坏死区，留下瓷样瘢痕。最被认可的发病机制理论基于内皮细胞损伤。该疾病尚无有效治疗方法。我们描述了一名26岁患有迪戈斯病的男性，诊断基于皮肤损害的临床和组织学表现。抗血小板治疗反应良好且8年随访期间无全身受累情况，这一点值得注意。我们认为该病例代表了该病的良性形式，通常称为良性皮肤迪戈斯病。
