Yellow nail syndrome (YNS) can induce bilateral exudative pleural effusion; however, to the best of our knowledge, no standard treatment for YNS has been established. The present study describes a patient with YNS for whom the pleural effusion was controlled by prednisolone. A 73-year-old man was referred to the University of Tsukuba Hospital (Ibaraki, Japan) complaining of shortness of breath, which was diagnosed as being due to bilateral pleural effusion. Based on the presence of yellowing and growth retardation of the toenails, lymphedema, bilateral exudative pleural fluid of unknown etiology, and lymphatic congestion on lymphoscintigraphy, the patient was diagnosed with YNS. The pleural fluid was predominantly lymphocytic and responded to systemic steroid administration [prednisolone 30 mg/day (0.5 mg/kg) for 2 weeks, with subsequent weekly tapering]. The general condition of the patient and their dyspnea also improved with treatment. These findings indicated that systemic steroid administration should be considered as one of the treatment options for patients with YNS who are reluctant to undergo chest drainage or pleurodesis due to the potential for a decrease in their ability to perform daily activities and respiratory function.

Primary lymphoedema, axillary web syndrome (AWS) and yellow nail syndrome may be related. Mr B is a 66-year-old gentleman with genital lymphoedema and lymphoedema of all four extremities. In 2023, he was diagnosed with non-Hodgkin lymphoma and also underwent cardiac surgery. In November 2023, he completed an inpatient rehabilitation at the Földi clinic in Germany, where he received intensive treatment for his lymphoedema and was also diagnosed with bilateral AWS. The presence of AWS in a patient with primary lymphoedema and no history of axillary surgery is unique. Although AWS typically presents after axillary surgery, this case highlights that it can also occur in patients without lymph node surgery. While the precise cause of this presentation of AWS is not known, it may be connected to yellow nail syndrome or potentially the recent chemotherapy treatment. This article will describe the clinical case, highlighting the need for further research on AWS present in primary lymphoedema.

Yellow nail syndrome is a rare condition occurring sporadically, with an extremely low prevalence rate. This syndrome classically presents with a triad of lower extremity edema, yellow nails, and mucosal issues such as pleural effusion and/or chronic sinusitis. Two out of the three features are deemed sufficient to diagnose a person with yellow nail syndrome. We present a rare case of yellow nail syndrome that began with chronic leg swelling and later progressed to the development of an asymptomatic pleural effusion and finally discoloration of nails. In our case, the patient did have a significant recent history of a total knee replacement with a titanium implant. Of note was the chronology of events including leg edema and asymptomatic pleural effusion which were present even before the titanium knee implant. The third feature of the hardening and yellow discoloration of the nails was found to have developed following the knee replacement. Interestingly, on further evaluation, he was found to have IgM deficiency.

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Recognizing and diagnosing the most common nail diseases is essential, to be able to guide patients and provide appropriate treatment. However, uncommon nail disorders should not be neglected, in order to avoid inadequate treatment and above all to ensure that no severe underlying disorder, with severe prognosis, is overlooked.

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.

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A 60-year-old man was referred to the outpatient clinic due to dyspnea of effort and productive coughing and rhinorrhea. Physical examination revealed swollen ankles and yellow, hyperkeratotic nails. HRCT showed bronchiectasis. This triad of symptoms indicates yellow nail syndrome. Vitamin E improved the yellow nails, while optimal expiration techniques alleviated respiratory symptoms.

A 60-year-old man was referred to the outpatient clinic due to dyspnea of effort and productive coughing and rhinorrhea. Physical examination revealed swollen ankles and yellow, hyperkeratotic nails. HRCT showed bronchiectasis. This triad of symptoms indicates yellow nail syndrome. Vitamin E improved the yellow nails, while optimal expiration techniques alleviated respiratory symptoms.

Yellow nail syndrome is a rare disease of unknown aetiology. Patients with YNS have a characteristic yellowish-coloured nails, pulmonary alterations and primary lymphedema. To the best of our knowledge, only a few reports of autopsy findings in these patients have been published. Its aetiology possibly involves a primary malformation of larger lymph vessels. We describe autopsy findings not previously associated with yellow nail syndrome, such as expansion of mediastinal lymph-nodes and splenic sinusoids. The present autopsy reveals hitherto unreported findings associated with YNS, such as alterations in splenic sinusoids and mediastinal lymph-node sinuses.