Faintuch J J, Saito R, Warth M do P, Rocha A dos S
Departamento de Clínica Médica, Faculdade de Medicina, Universidade de São Paulo.
Rev Hosp Clin Fac Med Sao Paulo. 1990 Sep-Oct;45(5):219-22.
A unusual case of a 61-year-old man who presented dyspnea and cough associated to skin and subcutaneous lesions typical of neurofibromatosis type 1 is reported. Chest roentgenograms and CT-scan showed emphysematous bullae. The patient underwent drainage of the greatest bulla, but death eventually ensured as a consequence of upper digestive bleeding. It can be expected that with the longer survival of neurofibromatosis patients with respiratory troubles, that are generally considered unusual will be recognized more frequently.
报告了一例61岁男性的罕见病例，该患者出现呼吸困难和咳嗽，并伴有1型神经纤维瘤病典型的皮肤和皮下病变。胸部X线片和CT扫描显示有肺气肿大疱。患者接受了最大的大疱引流，但最终因上消化道出血死亡。可以预期，随着患有呼吸问题的神经纤维瘤病患者生存期延长，通常被认为不常见的情况将更频繁地被识别出来。
