Bilateral axillary web syndrome in a patient with primary lymphoedema of upper limbs and non-Hodgkin lymphoma.

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: Analysis of 23 Consecutive Patients and Effect of Combined Fluconazole-Vitamin-E Treatment.

INTRODUCTION: 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. METHOD: 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.

[A man with yellow nails]. / Een man met gele nagels.

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. A case report with postmortem study]. / Síndrome de las uñas amarillas. Presentación de un caso con estudio autópsico.

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.

Cryptosporidiosis in Yellow Nail Syndrome - A Rare Case Report.

BACKGROUND: Cryptosporidium species infection causes malabsorption and severe diarrhea in immunocompromised hosts. Association of Yellow Nail Syndrome (YNS) and Cryptosporidiosis is rare and has not been reported till date. Immunity can also be affected in this case of YNS is associated with autoimmune disorders. CASE PRESENTATION: Here, we describe a case of persistent diarrhea in an 8 month old YNS patient. Modified Ziehl-Neelsen staining and Saffranine-Methylene blue revealed oocyts of Cryptosporidium species. Following appropriate treatment, the patient's symptoms improved and the patient was discharged in a hemodynamically stable condition. DISCUSSION: Cryptosporidiosis is a significant cause of morbidity and mortality in immunocompromised patients. YNS per se as well as treatment including steroids leads to immunosuppression in individuals making them susceptible host for opportunistic infections like Cryptosporidiosis. CONCLUSION: Clinicians should be aware of the condition and screen for Cryptosporidiosis in any immunocompromised patients with diarrheal symptoms, as parasitic infection like this are opportunistic in them.

Respiratory manifestation of yellow nail syndrome: a case report and literature review.

Yellow nail syndrome (YNS) is a rare disorder, and diagnosis is based on the clinical findings and the exclusion of other possible causes; the pathogenesis is poorly understood. YNS can be an isolated condition or associated with other diseases; however, YNS associated with multiple myeloma (MM) is rare. A 53-year-old male patient presented with coughing and shortness of breath, and he was diagnosed with YNS with MM. He underwent chemotherapy and achieved a good response. Although the etiology of YNS remains unknown, treating the underlying disease may help prevent or relieve the clinical signs.

A 49-Year-Old Woman With Persistent Bilateral Pleural Effusions.

CASE PRESENTATION: A 49-year-old woman presented to the ED with sudden onset abdominal pain, nausea, and vomiting. Her medical history included an uncomplicated gastric lap band surgery 9 years ago and subsequent removal of lap band after 6 years. She had a Roux-en-Y gastric bypass and cholecystectomy 5 months prior to the current presentation. The patient had been diagnosed with asthma and was prescribed an inhaled corticosteroid that she used only as needed. The patient denied smoking and heavy alcohol consumption. She was currently employed as a scrub technician in a local surgical center.

Yellow Nail Syndrome: A Case Presentation and a Review of Management Options.

Yellow nail syndrome (YNS) has traditionally been thought of as a triad of exudative pleural effusion, yellow nails, and lymphedema. More recently, in addition to the hallmark yellowish nail discoloration, the diagnostic criteria required an associated lymphedema and/or chronic respiratory manifestations including pleural effusions, bronchiectasis or chronic sinusitis. Etiology remains unknown and treatment is supportive and directed towards patient's specific complaints. While described alongside multiple endocrine, lymphatic and autoimmune disorders, its most ominous association is malignancy, raising YNS as a possible paraneoplastic condition. Here we present the case of an 80 years-old female with worsening restrictive airway disease and acquired yellow nails, with development of dyspnea, cough and leg edema. Recurrent exudative lymphocyte predominant pleural effusion was treated definitively with pleurodesis. Her leg edema and yellow nails were treated conservatively. We describe previous case reports and series in the literature, outline therapeutic options and discuss prognosis.

Yellow nail syndrome: a genetic Disorder?

BACKGROUND: Yellow nail syndrome is a very rare disorder. The classic clinical presentation includes a triad involving yellow nail discoloration, chronic pulmonary manifestations and lower limb lymphedema. Its etiology remains unknown and the genetic disorder is still controversial. OBSERVATION: We report a case of a young man diagnosed with this syndrome and for who the same affection was found in several members of his family Conclusion: The diagnosis of yellow nail syndrome in several members of the same family supports the fact that it is a genetic disorder.

Yellow Nail Syndrome Patients with Diffuse Panbronchiolitis-like Pulmonary Manifestation.

Yellow nail syndrome (YNS) is a rare clinical syndrome characterized by a triad of yellow thick nail, lymphedema and respiratory diseases. We experienced 2 cases of YNS with diffuse panbronchiolitis (DPB)-like pulmonary manifestation. Since YNS might be hidden to those who have been diagnosed with DPB, physicians should be alert to recognize nail signs of YNS in case of DPB refractory to macrolide therapy. We hereby review previous case reports of YNS and discuss its pulmonary manifestations.

Hyperplasia of Lymphoid Follicles and Lymphangiectasia in the Parietal Pleura in Bucillamine-induced Yellow Nail Syndrome.

Yellow nail syndrome (YNS) pleurisy is often difficult to control, and pathological examinations have rarely been reported. We herein report a case of bucillamine-induced YNS in which histopathology of the parietal pleura revealed hyperplasia of the lymphoid follicles and lymphangiectasia. Even after the discontinuation of bucillamine, the pleurisy and lymphedema showed no change. Based on the histopathological findings showing similarity to rheumatoid pleurisy, we administered corticosteroid treatments, and both the pleurisy and lymphedema improved. The findings in the present case suggest that, in bucillamine-induced YNS, pleurisy may be related to inflammation caused by rheumatoid arthritis in addition to abnormalities in lymphatic vessels.

Yellow Nail Syndrome Presenting With a Pericardial Effusion: A Case Report and Review of the Literature.

Yellow nail syndrome (YNS) is a constellation of clinical findings including at least 2 of the 3 features of thickened yellow nails, respiratory tract involvement, and lymphedema. We report the case of a middle-aged man presenting with dystrophic, thickened yellow nails; an idiopathic pericardial effusion in the absence of pleural effusion(s); and unilateral apical bronchiectasis found on computed tomography of the chest. This represents a unique presentation of YNS as the first report of a patient with YNS and a pericardial effusion in the absence of pleural effusions and lymphedema and is the 11th case report of YNS with pericardial effusion.

Yellow nail syndrome: a review.

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.

Yellow nail syndrome: a case report and review of treatment options.

BACKGROUND AND AIMS: Yellow nail syndrome is a rare disorder involving characteristic nail changes, lymphedema and chronic respiratory symptoms. Currently there is no definitive treatment and there have been no prospective randomised controlled trials evaluating the available options. In order to strengthen the literature on this topic, we present the case of a 67-year-old man with YNS and a detailed review of current treatment options. METHODS: We included 40 articles for the final review according to their relevance with the subject. RESULTS: Data for use of the commonly chosen therapies for YNS remains inconclusive, with small studies and case reports showing mixed results of efficacy. CONCLUSION: Although the date indicates that it is reasonable to recommend a trial of conservative therapy including vitamin E, antibiotics and compression stockings before pursuing more aggressive or invasive modalities, larger scale studies are required to determine the true efficacy of all treatment options.

Bronchiectasis in yellow nail syndrome.

BACKGROUND AND OBJECTIVE: Yellow nail syndrome (YNS) is a rare and poorly described disease process. In this case-control study, clinical features and findings on HRCT were compared with idiopathic bronchiectasis (IBx). METHODS: A review of all patients attending an adult bronchiectasis clinic between 2007 and 2013 identified 25 YNS patients. IBx patients were matched in a 2:1 ratio for age, duration of symptoms and gender. RESULTS: Median age of onset was 53 years. There were 12 male and 23 Caucasian YNS patients. Respiratory manifestations included chronic productive cough (100%), chronic rhinosinusitis (88%), pleural effusions (20%) and lymphoedema (12%). Chest symptoms preceded yellow nails in the majority (68%). Abnormal nails persisted at follow-up in 23 of 25 patients but improved in 14. In both disorders, there was symmetrical, predominantly lower lobe bronchiectasis on HRCT. Extent (P = 0.04), severity (P = 0.03) and bronchial wall thickness (P = 0.05) scores were lower in YNS, with less upper and middle lobe disease. Multivariate analysis showed an independent association with increased mucus plugging in YNS. There was a similar prevalence of Pseudomonas aeruginosa infection and mild lung function abnormalities. CONCLUSION: Bronchiectasis in YNS is less severe than IBx but is associated with increased mucus plugging, onset is in middle age and there is no female predominance. Treatment targeted at improved secretion clearance may improve both chest and nail symptoms, with consideration of long-term macrolide antibiotics.