Ganzoni A, Schneider J
Schweiz Med Wochenschr. 1981 Oct 3;111(40):1479-87.
Even severe generalized amyloidoses are difficult to diagnose clinically. By way of example the case is reported of a 64-year-old patient hospitalized for severe abdominal pain who died within a few weeks of heart and circulatory failure. The unusual disease picture is dominated by three cardinal symptoms: large, hemorrhagic-bullous lesions of the skin, recurrent ventricular ulcers with perforation and local peritonitis, and progressive heart failure refractory to therapy. Pathologico-anatomically, amyloid deposits were found in all three organs and primary systemic amyloidosis was diagnosed. Stenotic deposits in the blood vessels and extensive involvement of the ventricular wall suggest that the ulcer is a consequence of the primary disease. The cases of amyloidosis found at autopsy at the Institute of Pathology, Zurich, over the last seven years are reviewed.
即使是严重的全身性淀粉样变性在临床上也很难诊断。例如,报道了一名64岁因严重腹痛住院的患者,在几周内死于心脏和循环衰竭。这种不寻常的疾病表现主要由三个主要症状主导:皮肤出现大的出血性水疱病变、反复出现的心室溃疡伴穿孔和局部腹膜炎,以及对治疗无效的进行性心力衰竭。病理解剖学检查发现,所有三个器官均有淀粉样沉积物,诊断为原发性系统性淀粉样变性。血管中的狭窄沉积物和心室壁的广泛受累表明溃疡是原发性疾病的结果。本文回顾了苏黎世病理研究所过去七年尸检中发现的淀粉样变性病例。