Sinonasal mucosal vasculature in yellow nail syndrome and chronic rhinosinusitis with or without polyps.

BACKGROUND: Lymphatic dysfunction is hypothesized to be an underlying factor in the pathophysiology of yellow nail syndrome (YNS) (yellow nails, lymphedema, pleural effusions, and frequently chronic rhinosinusitis [CRS]). It is unclear why some YNS patients develop CRS. We quantified lymphatic and total vasculature of sinonasal mucosa in YNS patients and compared it to controls from CRS patients with (CRSwNP) or without polyps (CRSsNP). METHODS: Immunohistochemistry was performed on archival sinonasal mucosal samples from 5 patients with YNS and 14 controls with antibodies against podoplanin and CD31, markers of lymphatics. Morphometric assessment was performed on digital images using ImageJ software. RESULTS: In YNS, the number of lymphatics/mm2 ranged from 7 to 18/mm2 (controls: 6 to 43/mm2 , p = 0.343), with a mean perimeter between 92 and 201 µm (controls: 42 to 280 µm, p = 0.482). Total vasculature density was higher than lymphatics, ranging between 189 and 1159 vessels/mm2 , average 669 (controls: 139 to 1467/mm2 , average 503, p = 0.257) with smaller average perimeter, 40 to 117 µm, mean 64.8 µm (controls: 42 to 92 µm, mean 65.3 µm, p = 0.965). Lymphatics constituted only a small fragment of the total vasculature, ranging from 1.15% to 4.76%, average 2.34% (controls: 0.81% to 10.58%, average 4.88%, p = 0.156). CRSwNP patients had significantly higher lymphatic density (p = 0.011) and ratio of lymphatics to total vasculature (p = 0.045) than patients with YNS or CRSsNP. CONCLUSION: This is the first histological analysis of sinus mucosa in patients with YNS. Vascular type, density, size, and distribution in the sinonasal mucosa of YNS patients are not statistically significantly different from those of the CRSsNP group. Lymphatic density and ratio to total vasculature is higher in CRSwNP patients.

Hereditary Yellow Nail without a Syndrome.

A 40-year-old woman presented with clinical features of yellow nail syndrome (YNS). She had no past or present associated lymphatic or respiratory abnormalities. She was accompanied by her 12-year-old daughter, who also demonstrated clinical features of YNS. Similarly, the daughter had no past or present associated lymphatic or respiratory abnormalities. At the time of evaluation, a specific treatment was not initiated for mother or daughter. (SKINmed. 2019;17:73-74).

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.

Transient yellow discoloration of the nails for differential diagnosis with yellow nail syndrome.

A differential diagnosis must be made between transient yellow discoloration of the nails and yellow nail syndrome. We highlight some practical aspects of yellow nail discoloration.

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.

Fifty shades of yellow: a review of the xanthodermatoses.

The xanthodermatoses consist of a heterogeneous group of cutaneous disorders characterized by the macroscopic yellow hue seen on examination. This hue is attributable to the chemical structure of the accumulating substances within the skin or surrounding tissues. The most common culprits are lipids (cholesterol and triglycerides), elastin, and bilirubin. Exogenous sources of yellow pigment include yellow dyes (including hennas) and metal salts. This article will focus on recognition of these entities, classified in terms of morphology and the site of initial eruption, in order to support the recognition and diagnosis of these widely variable conditions.

Unusual manifestation of the yellow nail syndrome--case report.

The yellow nail syndrome is a rare disorder characterized by the classic triad of yellow and dystrophic nails, lymphedema and pleural effusion. We report in this paper a case of yellow nail syndrome, presenting the classic triad of the disease, associated with an unusual lymph accumulation in the abdomen region.

Unusual manifestation of the yellow nail syndrome - Case report

The yellow nail syndrome is a rare disorder characterized by the classic triad of yellow and dystrophic nails, lymphedema and pleural effusion. We report in this paper a case of yellow nail syndrome, presenting the classic triad of the disease, associated with an unusual lymph accumulation in the abdomen region.

A staged management of prolonged chylothorax in a patient with yellow nail syndrome.

Spontaneous chylothorax remains an unusual condition with diverse aetiologies and non-unified management strategies. Owing to the rarity of the condition, the evidence of management remains from case reports and limited retrospective studies. This case represents a difficult-to-manage chylothorax secondary to yellow nail syndrome with initial failure of both surgical and conservative treatment methods. Pleurovenous shunting represents a surgical management approach allowing the patient to live with and control an ongoing chyle leak.

[Pleuritis in yellow nail syndrome]. / Pleurabefunde beim Yellow-Nail-Syndrom.

A 76-year-old man presented clinically with coughing and shortness of breath and was diagnosed radiologically to have massive pleural effusion as a combined feature of yellow nail syndrome. A lung biopsy was taken and revealed histologically: chronic non-specific inflammation in the pleuropulmonary border, intrapleural edema with eightfold pleural thickening in comparison to normal, angiogenesis in both the nutritive and functional intrapleural blood vessels, no abnormalities of lymphatic vessels with normal topographical distribution as detected by immunohistochemistry for antibody D2-40, granulomatous chronic foreign body reaction as a consequence of pleural effusion therapy by talcum pleurodesis.The histopathological findings of chronic non-specific pleuritis with angiogenesis and increased permeability of blood vessels led to massive intrapleural edema with pleural effusion. Abnormalities of lymphatic vessels could not be confirmed. Considering the features of this disease, they are probably secondary to chronic r infectious or immunological inflammation or paraneoplastic complications with angiogenesis (in about 19%).

Congenital yellow nail syndrome: a case report and its relationship to nonimmune fetal hydrops.

Yellow nail syndrome (YNS) is an uncommon disorder characterized by a triad of nail dystrophy, lymphedema, and pleural effusion. It is rare in children and congenital occurrence of YNS has been very rarely described. We report a 2-year-old Arab boy having congenital yellow nail syndrome with mild facial dysmorphism and bilateral conjunctival pigmentation born to consanguineous parents. One of his older siblings had died of nonimmune fetal hydrops (NIFH). The case supports the genetic basis of yellow nail syndrome with a possible relationship to nonimmune fetal hydrops.

Yellow nail syndrome.

Yellow nail syndrome is an acquired condition of unknown etiology, rarely seen in children, characterized by a triad of thickened yellow nails, primary lymphedema, and respiratory manifestations. We report an 8-year-old girl with this syndrome who showed improvement with Fluconazole, 200 mg once weekly and vitamin E, 1000 IU once daily.

Yellow nails as an adverse reaction to the topical use of 5-fluorouracil for the treatment of nail psoriasis.

Three patients affected with nail psoriasis of the fingers in whom the use of topical 5-fluorouracil (5-FU) led to yellow nail discoloration are reported. Based on the fact that yellow nails are associated with a slow nail growth rate, nail growth in these patients ('bad responders') and in two individuals, both affected with psoriasis, who had previously responded favorably ('good responders') to topical 5-FU for their nail condition were compared. The results of nail growth measurement revealed that mean longitudinal growth in 'bad responders' was lower than that in 'good responders'. In conclusion, although topical application of 5-FU is an effective treatment for nail psoriasis, we suggest its use in those patients in whom nail growth is not significantly impaired. For that reason an observational period to evaluate nail growth prior to treatment with topical 5-FU would be an adequate means of patient recruitment.