Abstract：
BACKGROUND: Yellow nail syndrome (YNS), a very rare disorder of unknown etiology, is characterized by a triad associating yellow nails, respiratory manifestations, and lymphedema. YNS treatment remains non-codified.
METHODS: This retrospective study was conducted from January 2008 to December 2022 in a single tertiary department exclusively dedicated to lymphatic diseases. All consecutive patients with YNS were included.
RESULTS: Thirteen men and 10 women were included. Three patients had yellow nails at birth or during childhood. For the other 20 patients, median (Q1-Q3) age at first sign was 50.8 (43-61) years, with first-YNS-sign-to-diagnosis interval of 17 (10-56) months. For 4 patients, YNS was associated with primary intestinal lymphangiectasia. The first YNS sign was chronic cough (45.5%), followed by yellow nails (27.3%), chronic sinusitis (18.2%), and lymphedema (9.1%). At first consultation for all patients, 69.6% had the complete triad, all had yellow nails and cough, 82.6% had chronic sinusitis, and 69.6% had lymphedema. Twelve patients\' lymphedema involved only the lower limb(s), 2 the lower and upper limbs, and 2 the lower and upper limbs and face. Nineteen (82.6%) patients were prescribed fluconazole (100 mg/day [n = 8] or 300 mg/week [n = 11]) combined with vitamin E (1,000 mg/day) for a median of 13 months. Responses were complete for 4 (21.1%) patients, partial for 8 (42.1%), and therapeutic failures for 7 (36.8%).
CONCLUSIONS: YNS is a rare disease that almost always starts with a chronic cough. Despite inconstant efficacy, fluconazole-vitamin E in combination can be prescribed to treat yellow nails.
METHODS: This retrospective study was conducted from January 2008 to December 2022 in a single tertiary department exclusively dedicated to lymphatic diseases. All consecutive patients with YNS were included.
RESULTS: Thirteen men and 10 women were included. Three patients had yellow nails at birth or during childhood. For the other 20 patients, median (Q1-Q3) age at first sign was 50.8 (43-61) years, with first-YNS-sign-to-diagnosis interval of 17 (10-56) months. For 4 patients, YNS was associated with primary intestinal lymphangiectasia. The first YNS sign was chronic cough (45.5%), followed by yellow nails (27.3%), chronic sinusitis (18.2%), and lymphedema (9.1%). At first consultation for all patients, 69.6% had the complete triad, all had yellow nails and cough, 82.6% had chronic sinusitis, and 69.6% had lymphedema. Twelve patients\' lymphedema involved only the lower limb(s), 2 the lower and upper limbs, and 2 the lower and upper limbs and face. Nineteen (82.6%) patients were prescribed fluconazole (100 mg/day [n = 8] or 300 mg/week [n = 11]) combined with vitamin E (1,000 mg/day) for a median of 13 months. Responses were complete for 4 (21.1%) patients, partial for 8 (42.1%), and therapeutic failures for 7 (36.8%).
CONCLUSIONS: YNS is a rare disease that almost always starts with a chronic cough. Despite inconstant efficacy, fluconazole-vitamin E in combination can be prescribed to treat yellow nails.
摘要：
背景：黄指甲综合征（YNS），一种病因不明的非常罕见的疾病，特征是三合会关联黄色指甲，呼吸道表现和淋巴水肿。YNS治疗仍未编码。
方法：这项回顾性研究于2008年1月至2022年12月在专门致力于淋巴疾病的单个三级部门进行。包括所有YNS的连续患者。
结果：13名男性和10名女性被纳入研究。三名患者在出生时或童年时期有黄色指甲。对于其他20名患者，首次体征时的中位年龄（Q1-Q3）为50.8（43-61）岁。中位数（Q1-Q3）首次YNS体征至诊断间隔为17（10-56）个月。对于4名患者，YNS与原发性肠淋巴管扩张有关。第一个YNS体征是慢性咳嗽（45.5％），其次是黄色指甲(27.3%)，慢性鼻窦炎（18.2%）和淋巴水肿（9.1%）。在第一次咨询时,在这23名患者中，69.6%有完整的三合会，都有黄色的指甲和咳嗽，82.6%慢性鼻窦炎和69.6%淋巴水肿。淋巴水肿仅累及下肢（n=12），下肢和上肢（n=2），下肢和上肢和面部（n=2）。19例(82.6%)患者接受氟康唑(100mg/d,（n=8）或300毫克/周，（n=11））与维生素E（1000mg/d）联合使用，中位时间为13个月。4例（21.1％）患者的反应完成，部分8例（42.1%），治疗失败7例（36.8%）。
结论：YNS是一种罕见的疾病，几乎总是以慢性咳嗽开始。尽管疗效不稳定，氟康唑-维生素E组合可以用于治疗黄指甲。
方法：这项回顾性研究于2008年1月至2022年12月在专门致力于淋巴疾病的单个三级部门进行。包括所有YNS的连续患者。
结果：13名男性和10名女性被纳入研究。三名患者在出生时或童年时期有黄色指甲。对于其他20名患者，首次体征时的中位年龄（Q1-Q3）为50.8（43-61）岁。中位数（Q1-Q3）首次YNS体征至诊断间隔为17（10-56）个月。对于4名患者，YNS与原发性肠淋巴管扩张有关。第一个YNS体征是慢性咳嗽（45.5％），其次是黄色指甲(27.3%)，慢性鼻窦炎（18.2%）和淋巴水肿（9.1%）。在第一次咨询时,在这23名患者中，69.6%有完整的三合会，都有黄色的指甲和咳嗽，82.6%慢性鼻窦炎和69.6%淋巴水肿。淋巴水肿仅累及下肢（n=12），下肢和上肢（n=2），下肢和上肢和面部（n=2）。19例(82.6%)患者接受氟康唑(100mg/d,（n=8）或300毫克/周，（n=11））与维生素E（1000mg/d）联合使用，中位时间为13个月。4例（21.1％）患者的反应完成，部分8例（42.1%），治疗失败7例（36.8%）。
结论：YNS是一种罕见的疾病，几乎总是以慢性咳嗽开始。尽管疗效不稳定，氟康唑-维生素E组合可以用于治疗黄指甲。
