Willa-Craps C, Zala L B, Nievergelt H, Hunziker T, Braathen L R
Dermatological Clinic, Inselspital, University of Berne, Switzerland.
Dermatology. 1997;195(1):89-90. doi: 10.1159/000245702.
We describe a 34-year-old woman presenting with a 1-year history of asymptomatic, atrophic papules disseminated mainly on the trunk. The clinical features were characteristic of malignant atrophic papulosis, and the histopathologic features were confirmatory. The etiology of this rare condition, described for the first time in 1941, remains unknown. Also the pathogenesis is still controversial. A vascular disorder has been postulated by most authors, primary inflammatory or thrombotic vascular changes as well as primary endothelial proliferation being often mentioned in the literature. In the present case, the biopsy specimen of a recent efflorescence showed these three alterations all together, making it impossible to identify conclusively the primary event. Thrombosis, being already noticed in such early lesions, is likely to be of pathogenic importance. Rheological therapy as described in the literature may therefore be the most appropriate. In the present case, the lesions came spontaneously to a standstill without drug therapy.
我们报告一名34岁女性，有1年病史，主要表现为躯干散在分布的无症状萎缩性丘疹。临床特征符合恶性萎缩性丘疹病，组织病理学特征可确诊。这种于1941年首次被描述的罕见疾病的病因仍不清楚。其发病机制也仍存在争议。大多数作者推测这是一种血管疾病，文献中常提及原发性炎症或血栓性血管改变以及原发性内皮细胞增殖。在本病例中，近期皮疹的活检标本同时显示了这三种改变，因此无法最终确定原发性病变。在如此早期的病变中就已发现血栓形成，这可能具有致病重要性。因此，文献中描述的流变学疗法可能是最合适的。在本病例中，未经药物治疗，病变自行停止发展。
