Yellow Nail Syndrome or Diffuse Lymphatic Network Disease

Christu, Konstantinos A; Pastaka, Chaido; Papadopoulos, Dimitrios; Klimi, Eleni; Gourgoulianis, Konstantinos

doi:10.14712/18059694.2019.78

Cited by 9 publications

(4 citation statements)

References 13 publications

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“…‘Yellow nail syndrome’ is a well recognized although uncommon condition of nail dystrophy associated with lymphoedema 42 . The primary pathology is thought to be lymphatic dysfunction, but the cause for the underlying nail dystrophy is poorly understood 43–45 . Further work in this field may reveal more about this condition and shed light on the higher prevalence of nail disease found in other causes of lymphoedema such as LF.…”

Section: Discussionmentioning

confidence: 99%

Interdigital lesions and frequency of acute dermatolymphangioadenitis in lymphoedema in a filariasis-endemic area

McPherson¹,

Singh²,

Addiss³

et al. 2006

British Journal of Dermatology

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Section: Discussionmentioning

confidence: 99%

Interdigital lesions and frequency of acute dermatolymphangioadenitis in lymphoedema in a filariasis-endemic area

McPherson¹,

Singh²,

Addiss³

et al. 2006

British Journal of Dermatology

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“…Comprehensive literature review revealed intestinal lymphangiectasia is rarely reported in patients > 65 years old, and nearly all cases in the elderly occurred in intestinal lymphangiectasia secondary to Waldenstrom macroglobulinemia, [ 26 ] or genetic syndromes. [ 27 ] This case appears to be the oldest reported case of PIL, with another case reported in a 73-year-old patient. [ 28 ] This report extends the maximal age of presentation of PIL; this old age is notable because PIL generally presents in infants as a presumed congenital defect.…”

Section: Discussionmentioning

confidence: 90%

Case report of primary intestinal lymphangiectasia diagnosed in an octogenarian by ileal intubation and by push enteroscopy after missed diagnosis by standard colonoscopy and EGD

2018

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Rationale:Primary intestinal lymphangiectasia (PIL) is a rare, presumably congenital lesion that is usually diagnosed in patients < 3 years old, is rarely first diagnosed in adulthood, and when first diagnosed in adulthood typically presents with symptoms for many years. Although PIL is often identified by endoscopic abnormalities, it must be emphasized that the jejunoileum/distal duodenum must be intubated for diagnosis because the lesions are present in these regions. This work demonstrates that 1)-PIL can occur in an octogenarian; 2)-shows that the characteristic endoscopic findings are not found at colonoscopy without terminal ileal intubation; and 3)-may be missed at standard EGD without distal duodenal intubation.Diagnoses:A patient initially presented at age 83 with symptoms of watery diarrhea, abdominal distention, 5-Kg-weight-gain, and weakness for one month, and had typical clinical findings of PIL including chylous ascites, pleural effusions, bilateral pitting leg edema, hypoalbuminemia, borderline lymphopenia, hypovitaminosis-D, and hypocalcemia. Protein-losing-enteropathy was demonstrated by positive stool tests for alpha-1-antitrypsin. Standard colonoscopy revealed no significant lesions, but terminal ileal intubation during colonoscopy demonstrated creamy-white, punctate, mucosal lesions in terminal ileum, characteristic of lymphangiectasia. EGD with intubation to mid-descending duodenum revealed no significant lesions, but subsequent enteroscopy demonstrated lesions in distal duodenum/proximal jejunum similar to those in terminal ileum characteristic of lymphangiectasia. Histopathologic analysis of lesions of terminal ileum/distal duodenum demonstrated dilated mucosal vessels, confirmed as lymphatic vessels by immunohistochemistry. PIL was diagnosed after excluding secondary causes of intestinal lymphangiectasia.Interventions/Outcomes:Patient placed on standard PIL diet: oral supplements of medium-chain triglycerides, a high protein diet, supplements of fat-soluble vitamins, and avoiding long-chain fatty acids, with marked clinical improvement.Lessons:This work shows that: 1)-standard EGD and colonoscopy may miss characteristic lesions of PIL, 2)-enteroscopy or terminal ileal intubation at colonoscopy may be required for the diagnosis because lesions are typically located in distal duodenum/jejunoileum; and 3)-PIL can first present in the very elderly even with symptoms of short duration.

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“…One hundred and twelve studies involving 150 patients were selected for review, spanning a period of nearly 50 years. Figure presents a flow chart for the complete breakdown in the identification of appropriate studies . They all corresponded to the description of isolated case reports (between 1 and 4 per item) and a retrospective series of 19 patients…”

Section: Resultsmentioning

confidence: 99%

Characteristics of patients with yellow nail syndrome and pleural effusion

et al. 2014

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Yellow nail syndrome (YNS) can be associated with a pleural effusion (PE) but the characteristics of these patients are not well defined. We performed a systematic review across four electronic databases for studies reporting clinical findings, PE characteristics, and most effective treatment of YNS. Case descriptions and retrospective studies were included, unrestricted by year of publication. We reviewed 112 studies (150 patients), spanning a period of nearly 50 years. The male/female ratio was 1.2/1. The median age was 60 years (range: 0-88). Seventy-eight percent were between 41-80 years old. All cases had lymphoedema and 85.6% had yellow nails. PEs were bilateral in 68.3%. The appearance of the fluid was serous in 75.3%, milky in 22.3% and purulent in 3.5%. The PE was an exudate in 94.7% with lymphocytic predominance in 96% with a low count of nucleated cells. In 61 of 66 (92.4%) of patients, pleural fluid protein values were >3 g/dL, and typically higher than pleural fluid LDH. Pleurodesis and decortication/pleurectomy were effective in 81.8% and 88.9% of cases, respectively, in the treatment of symptomatic PEs. The development of YNS and PE occurs between the fifth to eighth decade of life and is associated with lymphoedema. The PE is usually bilateral and behaves as a lymphocyte-predominant exudate. The most effective treatments appear to be pleurodesis and decortication/pleurectomy.

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Yellow Nail Syndrome or Diffuse Lymphatic Network Disease

Abstract: We report a man aged 68 years old with pneumothorax and chronic bilateral pleural effusion in association with a history of yellow nails. The diagnosis of yellow nail syndrome based on yellow nails, lymphedema, chronic pleural effusion and intestinal lymphangiectasia.

Cited by 9 publications

References 13 publications

Interdigital lesions and frequency of acute dermatolymphangioadenitis in lymphoedema in a filariasis-endemic area

Interdigital lesions and frequency of acute dermatolymphangioadenitis in lymphoedema in a filariasis-endemic area

Case report of primary intestinal lymphangiectasia diagnosed in an octogenarian by ileal intubation and by push enteroscopy after missed diagnosis by standard colonoscopy and EGD

Characteristics of patients with yellow nail syndrome and pleural effusion

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