Yellow nail syndrome

By Dr Helen Mackie

Case
Sylvia, a 60-year-old woman, was referred to our lymphoedema clinic for assessment and management of widespread oedema of her limbs and face.

This has developed in the six months since a bout of severe bronchopneumonia, diagnosed as legionnaire’s disease.

On clinical examination, Sylvia’s chest sounds ‘noisy’ and a thick expectorant is noted. The oedema is firm and generalised, although worse in the feet and calves.

It is also noted that all of her fingernails have become thick and discoloured (see figure 1 below). Her toenails are similarly affected.

On investigation, a chest X-ray confirms a small pleural effusion and, compared with previous films, partially resolved pneumonia changes.

No fungal species were identified from culture of abnormal finger and toenail scrapings. Blood tests are unremarkable; in particular, renal function is normal.

Based on the presence of the nail changes combined with respiratory symptoms and lymphoedema, a diagnosis of yellow nail syndrome is made.


Figure 1: Fingernails have become thick and discoloured.

Discussion
Yellow nail syndrome is a rare disorder, first described in 1964, primarily recognised by the appearance of yellow nails and lymphoedema.1

Later, an association with respiratory illnesses was noted, and a triad for diagnosis was formed: yellow and thickened nails, lymphoedema and pleural effusion or other respiratory abnormality.

By 2009, about 150 cases had been reported in the literature.2

Male and female patients are equally affected, typically presenting between the fourth and sixth decades of life. Although very rare, paediatric cases have also been described.3

The pathogenesis of yellow nail syndrome is poorly understood. In most cases, the associated lymphatic dysfunction is considered to be acquired.

Infections may be a trigger, overwhelming an already dysfunctional lymphatic network.

The most common manifestation in yellow nail syndrome is abnormal yellow discolouration of the nails, with thickening, transverse ridging, excessive curvature, uneven pigmentation, diminished lunulae and onycholysis (see figure 2 above right and below).

Over time, these nail findings may vary in the same patient.

Lymphoedema is present in 80% of patients with yellow nail syndrome.4

It is pitting in type, typically involving the lower extremities symmetrically.

However, this lymphoedema may also occur in the upper extremities and face, as in Sylvia’s case. It can also affect the genitals and, occasionally, even occur in the peritoneal cavity where it will manifest as ascites.5

The respiratory manifestations of yellow nail syndrome are diverse, and include bronchiectasis and recurrent lower respiratory tract infections or chronic sinusitis in about 40% of patients.

Pleural effusions, usually bilateral, are seen in about 40% of cases, with chylothorax accounting for 30% of pleural effusions.4

As in Sylvia’s case, the diagnosis is clinical, based on the presence of the characteristic findings of abnormal nails, lymphoedema, and pleural effusions or other respiratory manifestations.

In the absence of another plausible explanation, two of the three manifestations of yellow nail syndrome is sufficient to consider the diagnosis.

In a Mayo Clinic series of 41 consecutive patients, the yellow nails eventually improved in about half the patients, often without specific nail-targeted treatment.6

Nail improvement appears to be associated with better control of respiratory and lymphatic manifestations.

The lymphoedema does tend to persist, although dramatic and durable improvements have been noted with decongestive treatment and compression therapy.

Therapeutic thoracocentesis may be required to control symptomatic pleural effusions, with pleurodesis considered for managing recurrent effusions.

The respiratory manifestations of bronchiectasis can be controlled with a combination of postural drainage and the judicious use of antimicrobial therapy.

Relatively little is known about the natural history of yellow nail syndrome, but the long-term prognosis appears generally favourable, especially with treatment of the clinical manifestations.


Figure 2: Note the thickening, transverse ridging, excessive curvature, uneven pigmentation, diminished lunulae and onycholysis. 

Outcome
In Sylvia’s case, an initial intensive program of complex lymphoedema therapy, including decongestive massage, bandaging of legs and exercise, resulted in control of leg lymphoedema in compression stockings and the resolution of arm lymphoedema.

Facial massage improved facial lymphoedema with relief of blocked sinuses.

Bronchiectasis slowly developed as a result of recurrent respiratory infections. Sylvia’s nails showed some improvement over time but appeared to worsen at times of respiratory deterioration.

The diagnosis of yellow nail syndrome caused Sylvia some bemusement that she would have a rare disease.

Although it did not relieve her from having to deal with a chronic multisystem disorder, it did make conversations with her treating medical professionals interesting.

Dr Helen Mackie is medical director of Mt Wilga Lymphoedema Service, Hornsby, NSW, and president of the Australian Lymphology Association.

References
1. British Journal of Dermatology 1964; 76:153-57.
2. Current Opinion in Pulmonary Medicine 2009; 15:371-75.
3. Canadian Respiratory Journal 2012; 19:35-36.
4. American Family Physician 2004; 69:1417-24.
5. American Journal of Respiratory and Critical Care Medicine 2012; 186:e10.
6. Chest 2008; 134:375-81.

This article first appeared on Australian Doctor on the 1st of April, 2014

Case Sylvia, a 60-year-old woman, was referred to our lymphoedema clinic for assessment and management...

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