Barabino A, Pesce F, Gatti R, Colotto P, Nobili F, Colacino R, Giampalmo A
III Paediatric Department G, Gaslini Scientific Institute, Genova, Italy.
Eur J Pediatr. 1990 Apr;149(7):457-8. doi: 10.1007/BF01959394.
A new case of malignant atrophic papulosis (Köhlmeier-Degos disease) is reported. Vascular symptoms began at 17 months of age with cerebral ischaemia and progressive involvement of fingers and toes with torpid ulcers and apical necrotic amputations. At 6 years of age he developed chronic intestinal ischaemia with malabsorption and a new cerebral attack; in spite of anti-aggregant therapy the disease progressed and he died 7 months after diagnosis from a third cerebral ictus. Since the typical skin lesions of the disease were absent, the diagnosis was made on the basis of a pathological pattern of an occluded biopsied artery. The elder brother presents clinical and instrumental vascular involvement without cutaneous lesions and could be slightly affected.
报告了一例新的恶性萎缩性丘疹病（科尔迈尔 - 德戈斯病）。血管症状始于17个月大时，表现为脑缺血，手指和脚趾逐渐受累，出现迟缓性溃疡和指尖坏死性截肢。6岁时，他出现慢性肠缺血伴吸收不良以及新的脑部发作；尽管进行了抗聚集治疗，疾病仍进展，诊断后7个月因第三次脑部发作死亡。由于该病典型的皮肤病变不存在，诊断是基于活检动脉闭塞的病理模式做出的。哥哥有临床和影像学血管受累，但无皮肤病变，可能受到轻微影响。
