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

El síndrome de las uñas amarillas es una enfermedad rara de etiología desconocida, caracterizado por una coloración amarillenta de las uñas, manifestaciones respiratorias y linfedemas primarios1. Existe poca literatura científica en referencia a estudios autópsicos de pacientes con este síndrome, así como respecto a su etiología, si bien se postula que es causada por una malformación de los conductos linfáticos. En este artículo presentamos el caso de una paciente diagnosticada en vida de síndrome de las uñas amarillas en cuyo estudio autópsico se encontraron algunos hallazgos previamente no descritos, como dilatación de los senos de ganglios linfáticos mediastinales y esplénicos.(AU)

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.(AU)

Yellow nail syndrome resulting from cardiac mitral valve replacement.

BACKGROUND: Yellow nail syndrome is a rare disease with unknown etiology, Attributed to functional anomalies or disturbance in lymphatic drainage. This condition is characterized by triad of nail discoloration, respiratory or intrathoracic manifestations and lymphedema. CASE PRESENTATION: Twenty days after mitral valve replacement for severe rheumatic mitral valve stenosis, 39 years old woman presented with face tenderness and hearing problems besides stuffy and clogged nose and underwent routin rhinosinusitis therapy. She came back to ears, nose and throat service with persistent rhinosinusitis as well as relapsing preoperative couphs and dyspnea besides lower extremities edema and toenails discoloration. After some modulations of treatment, she was introduced to pulmonary clinic on post -operative day = 30. Chest x ray showed a lot of left pleural effusion then she was returned to our service (cardiac surgery) on post- operative day = 33. The pigtail catheter was secured and we attained a significant amount of milky fluid which conformed with chylothorax. Finally Yellow nail syndrome was diagnosed with her on post-operative day = 35. Early conservative therapy such as bed rest, legs massage, low fat diet with medium chain triglycerides, diuretics, bronchodilator inhaler was not be able to satisfy us (chylous out put > 330 cc/d). Therefore the catheter replacement with chest tube was carried out followed by pleurodesis using Talc and doxycycline besides transition of oral intake to total parentral nutrition and vitamine E supplement, on post - operative day = 41. After that chylous leakage gradually subsided and patient was discharged to home on post- operative day = 47. At 4 weeks follow ups, chest x ray was clear without effusion and nails discoloration and legs lymphedema resolved. CONCLUSION: We reported the third post cardiac surgery Yellow nail syndrome which is an unclear entity with a set of associated signs and symptoms. Two prior reports involved with coronary artery bypass graft whereas we performed mitral valve replacement. In angiogram thoracic duct was not identified so that it seems post cardiac surgery Yellow nail the syndrome has iatrogenic origin due to the thoracic duct or its tributaries injury and requires meticulous assessment and management.

The color of skin: yellow diseases of the skin, nails, and mucosa.

The colors reflected from the skin are important indicators of dermatologic and systemic disorders. Incident light is subject to absorption by chromophores in the skin and scattering. Chromophores associated with yellow light reflection include the carotenoids and bilirubin. Various pathophysiologic mechanisms associated with these and other chromophores manifest with a yellow hue on examination. This review describes these mechanisms and the clinical features of yellow skin disorders by morphology. A brief summary of the differential diagnosis, laboratory investigations, and treatments are presented. Yellow skin disorders are a heterogenous group composed of abnormalities in keratin, elastic and connective tissue, lipid metabolism, and other states of metabolic, inflammatory, or organ dysfunction. Patients will present through different routes, and skin disease may precede or follow systemic disease. Dermatologists have an essential role in identifying those with malignant or systemic associations to ensure early diagnosis and treatment.

Yellow nail syndrome with chylothorax after coronary artery bypass grafting.

BACKGROUND: Yellow nail syndrome is a rare condition considered secondary to functional anomalies of lymphatic drainage. Yellow nail syndrome is diagnosed through the triad of intrathoracic findings (30% being pleural effusions), nail discoloration, and lymphedema, with any two features sufficient for diagnosis. We report the second case of post-operative yellow nail syndrome. CASE PRESENTATION: After coronary artery bypass grafting, our patient presented with chylothorax on post-operative day 13 and yellow toenail discoloration on post-operative day 28, diagnosing yellow nail syndrome. Initial conservative management with pigtail catheter drainage and low-fat diet with medium-chain triglycerides reduced chylous drainage from 350 mL/day on post-operative day 14 to < 100 mL/day on post-operative day 17. However, by post-operative day 18, drainage returned to 350 mL/day that persisted despite attempts to readjust the catheter position, replacement of catheter with chest tube, and transition to total parenteral nutrition and octreotide while nil per os. Lymphangiogram on post-operative day 32 did not identify the thoracic duct or cisterna chyli, precluding embolization. Talc and doxycycline pleurodeses performed on post-operative days 33 and 38, respectively, resolved his chylothorax and nail discoloration. CONCLUSIONS: Both yellow nail syndrome and chylothorax as a complication of coronary artery bypass grafting are rare entities. The proposed mechanism of post-operative chylothorax is iatrogenic injury to thoracic duct or collateral lymphatic vessels. Diagnosing yellow nail syndrome in patients with post-operative chylothorax (through co-existing yellow nail discoloration and/or lymphedema) may suggest predisposition to impaired lymphatic drainage, portending a difficult recovery and potentially indicating need for surgical management.

[Respiratory manifestations of yellow nail syndrome: report of two cases and literature review].

Objective: To describe the clinical characteristics of respiratory manifestations of yellow nail syndrome. Methods: We conducted a retrospective analysis of 2 patients with respiratory diseases associated with yellow nail syndrome. Their clinical and chest radiological data were collected. We searched PubMed, Wanfang and CNKI databases with the keywords "yellow nail syndrome, yellow nail and lung" in Chinese and English. And the relevant literatures, including 6 articles in Chinese and 81 articles in English, were reviewed. Results: Our 2 patients were male, one 60 years old and the other 76. Typical yellow nails were present in their fingers, and one of them also showed toe yellow nails. One patient was admitted for refractory respiratory infection and he was diagnosed with diffuse bronchiectasis. The respiratory symptoms could be relieved with antibiotics according to the results of sputum microbiological analysis. The other patient was admitted for cough and exertional dyspnea, and refractory pleural effusions were revealed bilaterally. He received repeated effusion drainage by thoracentesis, and Octreotide was tried recently. A total of 373 cases were reviewed in Chinese and English literatures. Pleural effusions (152 cases) and diffuse bronchiectasis (121 cases) were the most common reported respiratory manifestations. Lymphoedema was present in almost all cases with pleural effusion associated with yellow nail syndrome, and the effusion was usually exudative and lymphocyte predominant. Pleurodesis and decortication were effective for them. But, somatostatin analogues had been tried effectively for these patients recently. On the other hand, literatures showed that diffuse bronchiectasis in yellow nail syndrome was less severe than idiopathic diffuse bronchiectasis, and might benefit from long-term macrolide antibiotics. Conclusions: Yellow nail syndrome is a very rare disorder. Besides yellow nail, respiratory manifestations are the main clinical presentations. Diffuse bronchiectasis and recurrent pleural effusions are the common manifestations.

The Development of Yellow Nail Syndrome after the Implantation of a Permanent Cardiac Pacemaker.

Yellow nail syndrome (YNS) is characterized by yellowish nails, lymphedema, sinusitis, and pulmonary involvement and can be triggered by various underlying conditions, such as sinusitis or titanium exposure from an artificial joint or dental implant. Since YNS is potentially treatable, its timely diagnosis is important. The authors recently experienced a case of YNS in a patient who developed sinusitis, yellowish nails, bilateral edema of the extremities, and subclinical rheumatoid arthritis after the implantation of a cardiac pacemaker made from titanium. This case may be the first report of YNS induced by a titanium cardiac pacemaker.

Yellow nail syndrome after allogeneic haematopoietic stem cell transplantation in two patients with multiple myeloma.

OBJECTIVE AND IMPORTANCE: Yellow nail syndrome (YNS) is a rare disorder of unknown aetiology characterized by the triad of yellow nails, lymphoedema and respiratory manifestations. About 200 cases have been reported, but a lot of patients probably elude proper diagnosis because of both variability of symptoms and ignorance of this syndrome by many physicians. The pathogenesis remains unclear, and could involve functional lymphatic abnormalities, microvasculopathy or lymphocyte deficiency, but none of these hypotheses seems fully satisfactory. CLINICAL PRESENTATION: We report for the first time two cases of YNS associated with multiple myeloma relapsing after non-myeloablative haematopoietic cell transplantation (HCT). In these two cases, onset or worsening of YNS symptoms followed graft-versus-host disease (GvHD) manifestations. INTERVENTION: Corticosteroids given to treat GvHD also improved YNS manifestations. CONCLUSION: YNS after HCT might be a microvascular manifestation of endothelial GvHD and corticosteroids might be an effective treatment.

[Yellow nail syndrome: two pediatric case reports]. / Syndrome des ongles jaunes : deux cas pédiatriques.

BACKGROUND: Yellow nail syndrome (YNS) is a combination of specific nail dystrophy (yellowish or green nails, transverse ridging, increased curvature with "a hump" and distal onycholysis), lymphoedema and respiratory tract involvement (mainly bronchial hyper-responsiveness, recurrent pneumonia, bronchiectasis and pleural effusion), with or without sinusitis. This rare condition is most often seen in middle age. We report two pediatric cases. PATIENTS: A 9-year-old boy had 20 yellow nails and lymphedema of the lower and upper left limbs. He was born at 32 weeks of gestation. At birth, he had fetal hydrops with bilateral chylothorax. Since then, he presented bronchopulmonary dysplasia. In his first year of life, he had recurrent nasopharyngitis and serosal otitis. Lymphoscintigraphy was not performed. A 14-year-old boy had 10 yellow toenails and 2 yellow fingernails. He was born at 36 weeks of gestation with fetal hydrops. He had recurrent serosal otitis and asthma attacks. Lymphoscintigraphy showed lymphatic hypoplasia, especially of the left limbs. DISCUSSION: These two children have the classic triad of YNS, including yellow nails, lymphedema and respiratory tract involvement. Thirteen cases have been reported in children from birth to 10 years of age, three of whom were siblings, thus suggesting genetic predisposition. In most cases lymphoscintigraphy showed hypoplasia and/or ectasia of lymphatic vessels. These lymphatic abnormalities may be responsible for nail changes as seen in Turner's syndrome. The prognosis of YNS in adults may be serious because of the pulmonary tract involvement. However, it is not known in children. CONCLUSION: Yellow dystrophic nails in children may be associated with lymphedema and respiratory tract involvement, as in adults, and this condition should therefore be investigated in cases of YNS.

Yellow nail syndrome presenting with postpartum massive pleural effusion.

Yellow nail syndrome (YNS) is an uncommon clinical syndrome characterized by yellow-green discoloration of nails and recurrent respiratory tract lesions, pleural effusions and lymphedema. A 38-year-old woman was referred to the emergency complaining of chronic cough and increasing dyspnea within last 2 weeks. She had given birth 1 month ago. On examination, the patient exhibited dystrophic yellowish nails and mild peripheral lymphedema. A chest roentgenogram revealed a large right pleural effusion and a small left pleural effusion. YNS is a rare clinical entity but should be taken into consideration in patients with massive pleural effusions and persistent lymphedemas on the postpartum period.

Nail abnormalities associated with systemic pathologies.

Nail abnormalities can arise in conjunction with or as a result of systematic pathologies. These pathologies include single-organ diseases, multisystemic diseases, and drug-induced insults. Clinical signs associated with these conditions include dyschromias, vascular alterations, periungual tissue changes, textural dystrophies, contour alterations, and growth-rate alterations. The associated systemic pathologies may affect any part of the nail apparatus, including the nail matrix, the nail plate, the nail bed, the underlying vasculature, and the periungual tissues. The anatomical location and extent of damage determine the clinically manifested anomaly.

[Discovery of a yellow nail syndrome with major hypothyroidism]. / Découverte d'un syndrome des ongles jaunes lors d'une insuffisance thyroïdienne majeure.

The yellow nail syndrome is rare. It associates the triad: yellow nails, lymphedema and thoracic events. We report two cases of this syndrome with major hypothyroidism. These observations suggest an association between these two diseases.

Titanium, sinusitis, and the yellow nail syndrome.

Yellow nail syndrome is characterized by nail changes, respiratory disorders, and lymphedema. In a yellow nail patient with a skeletal titanium implant and with gold in her teeth, we found high levels of titanium in nail clippings. This study aims to examine the possible role of titanium in the genesis of the yellow nail syndrome. Nail clippings from patients with one or more features of the yellow nail syndrome were analyzed by energy dispersive X-ray fluorescence. Titanium was regularly found in finger nails in patients but not in control subjects. Visible nail changes were present in only half of the patients. Sinusitis with postnasal drip and cough was the most common complaint. The dominant source of titanium ions was titanium implants in the teeth or elsewhere. The titanium ions were released through the galvanic action of dental gold or amalgam or through the oxidative action of fluorides. In other patients the titanium was derived from titanium dioxide in drugs and confectionary. Stopping galvanic release of titanium ions or canceling exposure to titanium dioxide led to recovery. In one patient with a titanium implant, the symptoms recurred after renewed exposure to titanium. Yellow nail syndrome is caused by titanium.

Update on uncommon pleural effusions.

Although infections, malignancies and heart failure are responsible for the majority of pleural effusions, there are many other causes and several uncommon but distinctive types of pleural fluid. For this update we have chosen several uncommon forms of pleural effusions or disorders in which there have been recent advances in our understanding over the past several years. Chylothorax, pseudochylothorax and urinothorax are associated with characteristic clinical contexts and pleural fluid parameters but are likely underdiagnosed. Yellow nail syndrome is a rare disorder that can be associated with chylothorax and manifests multisystem features. Recognition of these entities is important because each of these disorders is associated with distinctive aetiology and management modalities. Correct diagnosis depends on the clinician's awareness of the clinical contexts and manifestations along with diagnostic pleural fluid findings in these disorders.

Yellow nail syndrome following thoracic surgery: a new association?

An 80-year-old man presented with the characteristic triad of yellow nail syndrome (chronic respiratory disorders, primary lymphedema and yellow nails) in association with coronary artery bypass graft surgery. Treatment with mechanical pleurodesis and vitamin E resulted in near complete resolution of the yellow nails, pleural effusions, and lower extremity edema. The etiology of the yellow nail syndrome has been described as an anatomical or functional lymphatic abnormality. Several conditions have previously been described as associated with this disease. This is the first report of the association of this syndrome with thoracic surgery.