Voigt C, Aumiller J, Saeger W, Kutzner H, Ostendorf P C
Abteilung Innere Medizin I, Marienkrankenhaus, Hamburg.
Dtsch Med Wochenschr. 1994 Oct 21;119(42):1427-30. doi: 10.1055/s-2008-1058856.
A 53-year-old woman had noticed numerous papules on her trunk and proximal parts of the limbs. They had healed with a central scar surrounded by a reddish wall. Four months later she had to undergo laparotomy for ileus, resulting from a perforation in the region of the middle jejunum: it was excised and sutured over. At laparotomy white plaque-like lesions were noted on the serosa of both small and large intestine. Histological examination of the jejunal exudate and of a skin biopsy both demonstrated malignant atrophic papulosis (Köhlmeier-Dégos' disease). Treatment with ticlopidine, a platelet-aggregation inhibitor (250 mg twice daily), was initiated and she has now been free of extracutaneous symptoms for 6 years.
一名53岁女性注意到其躯干及四肢近端出现大量丘疹。这些丘疹愈合后中央留有瘢痕，周围有红色边缘。四个月后，她因空肠中段穿孔导致肠梗阻而接受剖腹手术：穿孔部位被切除并缝合。剖腹手术时发现小肠和大肠浆膜上有白色斑块样病变。空肠渗出物及皮肤活检的组织学检查均显示为恶性萎缩性丘疹病（科尔迈尔-德戈斯病）。开始使用血小板聚集抑制剂噻氯匹定（每日两次，每次250毫克）进行治疗，目前她已无皮肤外症状达6年。
