Burg G, Vieluf D, Stolz W, Landthaler M, Braun-Falco O
Dermatologische Klinik und Poliklinik, Julius-Maximilians-Universität Würzburg.
Hautarzt. 1989 Aug;40(8):480-5.
On the basis of 106 patients with malignant atrophic papulosis in the literature, including one case of our own, the clinical, histopathological and nosological features of this disease are reviewed. Histopathologically, the typical papules with central porcelain-white atrophy show broad wedge-shaped necrosis of the connective tissue due to thrombotic occlusions of small vessels in the corium. The disease leads to death in 50% of these cases, mostly due to involvement of the gut and the central nervous system. Malignant atrophic papulosis is the prototype of a disease, in which skin lesions are the primary sign of a systemic disorder demanding the interdisciplinary cooperation of various specialists.
基于文献报道的106例恶性萎缩性丘疹病患者（包括我们自己诊治的1例），对该疾病的临床、组织病理学及疾病分类学特征进行了综述。组织病理学上，典型的中央呈瓷白色萎缩的丘疹显示真皮内小血管血栓性闭塞导致结缔组织广泛楔形坏死。该疾病在50%的病例中会导致死亡，主要是由于肠道和中枢神经系统受累。恶性萎缩性丘疹病是一种疾病的原型，其中皮肤损害是一种全身性疾病的主要体征，需要各专科进行跨学科合作。
