A Case-Control Study of the Lymphatic Phenotype of 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

Regional distribution of epifascial swelling and epifascial lymph drainage rate constants in breast cancer-related lymphedema.

The view that breast cancer-related lymphedema (BCRL) is a simple, direct mechanical result of axillary lymphatic obstruction ('stopcock' mechanism) appears incomplete, because parts of the swollen limb (e.g., hand) can remain nonswollen. The lymph drainage rate constant (k) falls in the swollen forearm but not in the spared hand, indicating regional differences in lymphatic function. Here the generality of the hypothesis that regional epifascial lymphatic failure underlies regional swelling was tested. To do so, the regional distribution of epifascial swelling along the forearm was compared with that of epifascial (subcutis) k