A Case-Control Study of the Lymphatic Phenotype of Yellow Nail Syndrome

作者：Cousins Emma; Cintolesi Viviana; Vass Laurence; Stanton Anthony W B; Irwin Andrew; Heenan Susan D; Mortimer Peter S

来源：Lymphatic Research and Biology, 2018, 16(4): 340-346.

DOI：10.1089/lrb.2018.0009

• lymphatic dysfunction
• lymphedema
• lymphoscintigraphy
• phenotype
• yellow nail syndrome

Background: Yellow nail syndrome (YNS) is a rare disease manifesting as a triad of yellow-green dystrophic nails, lymphedema, and chronic respiratory disease. The etiology of YNS is obscure and investigations are few. A single lymphatic pathogenesis has been proposed to account for all the associated features, and despite the lack of evidence for a unifying lymphatic mechanism, this hypothesis prevails. The objective was to explore the lymphatic phenotype in YNS and to establish whether lymphatic dysfunction could be a major contributing factor to the disease process.
Methods and Results: Four-limb lymphoscintigraphy was performed on patients with YNS and on healthy, age-matched controls. All 17 patients had lower limb swelling, and 14 (82%) had upper limb swelling also, including 5 (29%) with hand involvement. None of the YNS lymph scans was completely normal. Combined qualitative and quantitative assessment showed that 67% of YNS scans were clearly abnormal compared with 36% of healthy control scans. Mean axillary and ilio-inguinal nodal tracer uptakes were 41%-44% lower in the YNS group than in the controls (p < 0.0001).
Conclusions: YNS is a lymphatic phenotype because lymphatic insufficiency was found to exist in all patients and the insufficiency was widespread (upper and lower limbs), with a common mechanistic fault of poor transport. The origin of the lymphatic fault is unclear. In healthy individuals, lymphatic abnormalities may be relatively common in the fifth decade of life onward.

• 出版日期2018-8