Yellow nail syndrome

Last revised by Dr Rohit Sharma on 27 Apr 2020

The yellow nail syndrome (YNS) is a rare disorder principally affecting the lymphatic system.

It is characterized by a clinical triad:

  1. nail discolouration (chromonychia): yellow to dark green slow-growing dystrophic nails (scleronychia) 9
  2. lymphedema (peripheral/primary)
  3. pulmonary disease: see yellow nail syndrome (pulmonary manifestations)

Other associated features include:

It is a rare disease with an approximate incidence of less than one million/year 8. There may be a slightly increased female predilection 5. While clinical onset varies from birth to late adult life, it is thought to typically affect those in early middle age 5.

The syndrome can sometimes be associated with 

Clinical features tend to be variable in severity amongst affected individuals and temporal variation is common. 

The exact pathogenesis is not well understood although anatomical or functional lymphatic drainage abnormalities have been proposed as an underlying mechanism.

Imaging is often performed in the evaluation of pulmonary manifestations.

On CT chest, while individual features are non-specific, there may be evidence of

While there is no specific treatment for the syndrome, however some patients are helped with biotin and vitamin E supplementation 8. In addition most patients are often managed with supportive measures targeted at ameliorating the various clinical manifestations (e.g. treating respiratory infections).

The general clinical course is benign but is somewhat dependent on disease associations. In 10-30% cases there is spontaneous resolution of the condition 8.

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