Ayed Della S, Kotti A, Ben Sik Ali H, Ayed S, Fekih Hassen M, Elatrous S
Service de réanimation médicale, EPS Taher Sfar, 5100 Mahdia, Tunisie.
Rev Pneumol Clin. 2012 Jun;68(3):202-4. doi: 10.1016/j.pneumo.2011.08.001. Epub 2011 Oct 13.
The Von Recklinghausen disease is a genetic hereditary neurofibromatosis. It causes neurofibroma, axillary and inguinal lentigines, and café-au-lait spots in the skin. It may affect the lung in 5 to 20% of cases, causing neurofibroma, infiltrative and cystic lesions, emphysematous or bubble injury leading to a chronic respiratory failure. The risk of pneumothorax in theses cases seems higher. Few reviews reported the pulmonary manifestations in the Recklinghausen disease and specially the pneumothorax as a complication while the direct relation between this neurofibromatosis and the lung disease is not clearly established yet. We report a case report of spontaneous pneumothorax with slow evolution complicating the course of a patient with Recklinghausen disease.
冯·雷克林豪森病是一种遗传性神经纤维瘤病。它会导致神经纤维瘤、腋窝和腹股沟雀斑以及皮肤牛奶咖啡斑。在5%至20%的病例中，它可能会影响肺部，导致神经纤维瘤、浸润性和囊性病变、气肿或气泡损伤，进而导致慢性呼吸衰竭。这些病例中气胸的风险似乎更高。很少有综述报道雷克林豪森病的肺部表现，特别是作为并发症的气胸，而这种神经纤维瘤病与肺部疾病之间的直接关系尚未明确确立。我们报告一例自发性气胸伴缓慢进展的病例，该病例使一名患有雷克林豪森病的患者病情复杂化。
