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[恶性萎缩性丘疹病(科尔迈尔-德戈斯病):诊断、治疗及病程]

[Malignant atrophic papulosis (Köhlmeier-Degos): diagnosis, therapy and course].

作者信息

Vicktor C, Schultz-Ehrenburg U

机构信息

Dermatologische Klinik des Klinikum Buch, Berlin.

出版信息

Hautarzt. 2001 Aug;52(8):734-7. doi: 10.1007/s001050170092.

DOI:10.1007/s001050170092
PMID:11544947
Abstract

A 71-year-old male patient presented with malignant atrophic papulosis (Köhlmeier-Degos disease). He developed multiple distinctive cutaneous lesions. The histopathological findings showed an obliterated arteriole with a wedge-shaped area of ulcerated and necrobiotic dermis. Laboratory tests were within normal limits or not specific. Although the involvement of the gastrointestinal tract and other organs has been noted in approximately 60% of the reported cases, in this patient so far the skin seems to be the only involved site. There is no proven effective therapy for Köhlmeier-Degos disease. In our case treatment with pentoxifylline and aspirin led to healing of all skin lesions within 3 months. One year after beginning treatment, the patient showed neither new cutaneous lesions, nor any signs of systemic involvement. The combination of pentoxifylline and aspirin should be considered when planning treatment strategies for malignant atrophic papulosis.

摘要

一名71岁男性患者被诊断为恶性萎缩性丘疹病(科尔迈尔-德戈斯病)。他出现了多处独特的皮肤病变。组织病理学检查结果显示小动脉闭塞,伴有楔形的溃疡和坏死性真皮区域。实验室检查结果在正常范围内或不具有特异性。尽管在大约60%的报告病例中已注意到胃肠道和其他器官受累,但在该患者中,目前皮肤似乎是唯一受累部位。对于科尔迈尔-德戈斯病,尚无经证实有效的治疗方法。在我们的病例中,使用己酮可可碱和阿司匹林治疗后,所有皮肤病变在3个月内愈合。开始治疗一年后,患者既未出现新的皮肤病变,也没有任何全身受累的迹象。在制定恶性萎缩性丘疹病的治疗策略时,应考虑己酮可可碱和阿司匹林的联合使用。

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Degos disease - malignant atrophic papulosis or cutaneointestinal lethal syndrome: rarity of the disease.德戈斯病——恶性萎缩性丘疹病或皮肤肠道致命综合征:该疾病的罕见性。
Clin Exp Gastroenterol. 2015 Apr 16;8:141-7. doi: 10.2147/CEG.S59794. eCollection 2015.
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Malignant atrophic papulosis (Köhlmeier-Degos disease) - a review.
恶性萎缩性丘疹病(Köhlmeier-Degos 病)——综述。
Orphanet J Rare Dis. 2013 Jan 14;8:10. doi: 10.1186/1750-1172-8-10.