关键词: Lymphedema Respiratory manifestations Review Sinusitis Yellow nail syndrome

Mesh : Humans Lung Diseases / etiology pathology Lymphedema / etiology pathology Sinusitis / etiology pathology Yellow Nail Syndrome / complications diagnosis pathology

来  源:   DOI:10.1186/s13023-017-0594-4

Abstract:
Yellow nail syndrome (YNS; OMIM 153300, ORPHA662) is a very rare disorder that almost always occurs after 50 years of age but a juvenile or familial form has also been observed. YNS is diagnosed based on a triad associating yellow nail discoloration, pulmonary manifestations (chronic cough, bronchiectasia, pleural effusion) and lower limb lymphedema. Chronic sinusitis is frequently associated with the triad. YNS etiology remains unknown but a role of lymphatic impairment is usually evoked. YNS is more frequently isolated but may be associated in rare cases with autoimmune diseases, other clinical manifestations implicating lymphatic functions or cancer and, hence, is also considered a paraneoplastic syndrome. YNS management is symptomatic and not codified. YNS can resolve spontaneously. Oral vitamin E alone or even better when associated with triazole antifungals may achieve partial or total disappearance of nail discoloration. Pleural effusion can be treated surgically, with decortication/pleurectomy or pleurodesis. Antibiotic prophylaxis is prescribed for bronchiectasia with chronic sputum production. Lymphedema treatment is based on low-stretch bandages and the wearing of elastic compression garments combined with skin care, exercises and, as needed, manual lymph drainage.
摘要:
黄甲综合征(YNS;OMIM153300,ORPHA662)是一种非常罕见的疾病,几乎总是在50岁以后发生,但也观察到青少年或家族形式。YNS是根据与黄色指甲变色相关的三合会诊断的,肺部表现(慢性咳嗽,支气管扩张症,胸腔积液)和下肢淋巴水肿。慢性鼻窦炎通常与三联征有关。YNS的病因尚不清楚,但通常会引起淋巴损伤。YNS更常被隔离,但在罕见的自身免疫性疾病病例中可能与此相关。涉及淋巴功能或癌症的其他临床表现,因此,也被认为是副肿瘤综合征。YNS管理是有症状的,没有编纂。YNS可以自发解决。单独口服维生素E或甚至更好地与三唑抗真菌剂联合使用时,可以实现指甲变色的部分或全部消失。胸腔积液可以手术治疗,进行去胸膜/胸膜切除术或胸膜固定术。抗生素预防用于慢性痰产生的支气管扩张症。淋巴水肿治疗基于低弹力绷带和穿着弹性压缩服装结合皮肤护理,练习和,根据需要,手动淋巴引流。
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