Schneider A, Tschumi A, Egloff B, Ott F, Fanconi A
Helv Paediatr Acta. 1986 Dec;41(5):447-54.
The clinical and histological diagnosis of Degos' syndrome could be made in a 8-month-old infant with scarring skin lesions. Some weeks later the baby developed severe intestinal symptoms and died from perforations of multiple necrotic lesions of the small bowel. Autopsy revealed in addition to the skin and gut lesions a haemorrhagic cortical infarction of the brain and fibrotic areas in the myocardium. In all these lesions characteristic stenotic endovascular proliferations were seen. Malignant atrophic papulosis is an extremely rare disease in infancy (our patient is the fifth reported case) with a well defined clinical picture, characteristic histological appearance of obliterating vasculitis, but unknown aetiology.
Degos综合征的临床和组织学诊断可在一名患有瘢痕性皮肤病变的8个月大婴儿中做出。几周后，婴儿出现严重的肠道症状，并死于小肠多处坏死性病变穿孔。尸检显示，除皮肤和肠道病变外，还有脑皮质出血性梗死和心肌纤维化区域。在所有这些病变中均可见特征性的狭窄性血管内增生。恶性萎缩性丘疹病在婴儿期极为罕见（我们的患者是第五例报告病例），具有明确的临床表现、闭塞性血管炎的特征性组织学表现，但病因不明。
