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.

[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.

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.

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.

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.

An 80-Year-Old Man With Dyspnea and Bilateral Pleural Effusions After Partial Nephrectomy for Renal Cell Carcinoma.

An 80-year-old man presented because of superficial head trauma sustained after falling from bed. On review of systems, he reported worsening dyspnea on exertion, nonproductive cough, and weight loss over the preceding 2 to 3 months. There was no report of chest pain or leg swelling. He had a past medical history of hypertension, coronary artery disease, subclinical hypothyroidism, and renal cell carcinoma treated with partial right nephrectomy approximately 1 year before this presentation. Two months earlier he had been evaluated in the dermatology clinic for painful, dystrophic fingernails. At that time he was diagnosed with acropachy with onycholysis and suspected superinfection, and after failing to improve with vinegar soaks and topical antimicrobials, he underwent surgical nail removal on the second and fourth digits of the right hand. Histological examination of the operative specimens revealed dystrophic nails with negative fungal stains. His medications included levothyroxine, hydrochlorothiazide, and clopidogrel. He had never smoked and had done clerical work until retirement. He was originally from Colombia.

Thoracoscopy under Local Anesthesia was Useful for Diagnosing Yellow Nail Syndrome.

An 80-year-old woman with rheumatoid arthritis and a past history of tuberculosis presented with exertional dyspnea and edema of both legs. Chest X-ray performed on admission showed bilateral pleural effusion. Thoracoscopy under local anesthesia was performed, and vasodilation and non-specific yellowish inflammatory changes were noted in the pleura. A pathological examination showed chronic fibrosing pleuritis in addition to chronic pleural inflammatory changes with lymphoid aggregates. The nails on all fingers and toes were thickened and displayed yellow discoloration, and the edema was resistant to diuretics. Lymphoscintigraphy was conducted, which showed lymphatic drainage abnormalities. The patient was ultimately diagnosed as having yellow nail syndrome.

Aetiology of bronchiectasis in Guangzhou, southern China.

BACKGROUND AND OBJECTIVE: Aetiologies of bronchiectasis in mainland China and their comparisons with those in western countries are unknown. We aimed to investigate bronchiectasis aetiologies in Guangzhou, southern China, and to determine ethnic or geographic differences with reports from western countries. METHODS: Consecutive patients with steady-state bronchiectasis were randomly recruited. Past history was meticulously extracted. Patients underwent physical examination, saccharine test, humoral immunity assays, gastroesophageal reflux scoring and sputum culture. Fiberoptic bronchoscopy, total immunoglobin E (IgE) and Aspergillus fumigatus-specific IgE measurement, 24-h gastroesophageal pH monitoring and miscellaneous screening tests were performed, if indicated. This entailed comparisons on aetiologies with literature reports. RESULTS: We enrolled 148 patients (44.6 ± 13.8 years, 92 females), most of whom had mild to moderate bronchiectasis. Idiopathic (46.0%), post-infectious (27.0%) and immunodeficiency (8.8%) were the most common aetiologies. Miscellaneous aetiologies consisted of asthma (5.4%), gastroesophageal reflux (4.1%), aspergillosis (2.7%), congenital lung malformation (2.0%), Kartagener syndrome (1.4%), rheumatoid arthritis (1.4%), chronic obstructive pulmonary disease (0.7%), Young's syndrome (0.7%), yellow nail's syndrome (0.7%), eosinophilic bronchiolitis (0.7%) and foreign bodies (0.7%). No notable differences in clinical characteristics between idiopathic and known aetiologies were found. Ethnic or geographic variations of aetiologies were overall unremarkable. CONCLUSIONS: Idiopathic, post-infectious and immunodeficiency constitute major bronchiectasis aetiologies in Guangzhou. Clinical characteristics of patients between known aetiologies and idiopathic bronchiectasis were similar. Ethnicity and geography only account for limited differences in aetiologic spectra. These findings will offer rationales for early diagnosis and management of bronchiectasis in future studies and clinical practice in China.

[Enigmatic lymphatic diseases involving the lung]. / Pathologies énigmatiques des lymphatiques comportant une atteinte pulmonaire.

Lymphedema associated with other developmental malformations (Milroy syndrome, Hennekam syndrome, Noonan syndrome, Gorham-Stout syndrome, yellow nail syndrome) are unfrequent disease, but explorations led to the identification of genetic mutations that have then been validated in mouse models. However, lymphatic vessels complexity and its proximity with the venous system suggest the need for further researches, especially in the comprehension of pulmonary symptoms.

Atypical presentation of congenital yellow nail syndrome in a 2-year-old female.

BACKGROUND: Yellow nail syndrome (YNS) is a rare clinical entity of unknown etiology that is characterized by a triad of yellow nails, respiratory manifestations, and lymphedema. The condition appears in the mid- to later years of life and only rarely in childhood. We describe a rare case of YNS with an atypical clinical presentation consisting of only yellow and dystrophic nails in a 2-year-old female since birth. OBJECTIVE: A case of congenital YNS with only dystrophic and yellow nails is reported. METHODS AND RESULTS: A 2-year-old female presented with yellow nails since birth. There was no positive family history. Physical examination revealed 20 thickened, dystrophic, yellow nails with onycholysis. There was no evidence of respiratory manifestations or lymphedema. CONCLUSION: Although rare, YNS can present as a congenital clinical entity and persist after birth. Pediatric patients with YNS show different clinical manifestations than the classic adult patient. The presence of yellow and dystrophic nails in the absence of respiratory and lymphatic manifestations may be the only sign of pathology and warrants close monitoring as progression to more serious complications can occur.

A 24 year-old male with yellow nail syndrome having bronchiactasis without pleural effusion as a pulmonary manifestation.

Yellow nail syndrome is a rare entity of unknown etiology. Classically, it consists of a triad of slow-growing yellow nails of fingers and toes, lymphedema, and pulmonary manifestations mainly pleural effusion. Other pulmonary manifestations also have been described in the literature such as bronchiectasis, recurrent pneumonias, bronchitis, and sinusitis. This paper describes a case of yellow nail syndrome which did not have the classic triad of the condition; he presented with progressive yellow hand and toe nails intermittent lymphedema, bronchctasis, and sinusitis without pleural effusion.

Surgical treatment of congenital lymphedema.

Lymphedema is a pathologic condition that results from a disturbance of the lymphatic system, with localized fluid retention and tissue swelling. Primary lymphedema is a congenital disorder, caused by a malformation of lymph vessels or nodes. Major progress has been achieved in the radiologic diagnosis of patients affected by lymphedema. The ideal treatment of the affected limb should restore function and cosmetic appearance. Surgical treatment is an alternative method of controlling chronic lymphedema. Free lymph nodes autologous transplantation is a new approach for lymphatic reconstruction in hypoplastic forms of primary lymphedema. The transferred nodes pump extracellular liquid out of the affected limb and contain germinative cells that improve immune function.