Yellow nail syndrome: treatment with octreotide

The yellow nail syndrome (YNS) is the triad of 'yellow' nails, peripheral oedema and pleural effusions. For diagnosis, which is clinical, at least two of these findings are necessary. Also typical is a long-standing chronic cough often caused by low-grade bronchiectases. The pleural effusions often require pleurodesis. The pathogenesis is probably a dysfunction of the lymphatic system (1, 2). Octreotide regulates the release of growth hormone and thyrotropin, and also has effects on the gastro-intestinal tract, where it inhibits glandular secretion, neurotransmission, smooth-muscle contraction and absorption of nutrients. Adverse effects are nausea, abdominal cramps, diarrhoea, malabsorption of fat and flatulence (3). Because of the inhibition of absorption of fats and other nutrients, octreotide has been useful in chylothorax from many different causes (4). The pleural effusion in YNS is usually an exudate, but in rare cases a frank chylothorax. One such case with successful octreotide treatment has been described in the literature (5).

The aim of this report was to investigate the effect of octreotide treatment on a patient with YNS with pleural exudates not resulting from chylothorax.

A 62-year-old man with typical YNS presented with bilateral large pleural effusions (Fig. 1). He had suffered from repeated pneumonia for many years, and 10 years earlier mild bronchiectases were diagnosed and yellow nails were noted. From the right pleura, 1750-mL clear yellowish fluid was removed and a few days later, 1300 mL was removed from the left side. During the next few weeks, repeated thoracocenteses on both sides were necessary for the palliation of his dyspnoea, and the total amount of removed fluid was more than 10 L. The pleural fluid showed a low cholesterol value, 1.2 mmol/L (serum, 3.5), a fairly high albumin level, 19.0 g/L [serum, 25 g/L (normal, 36-45)], and no triglycerides. Octreotide was administered, initially 0.5 mg subcutaneously twice daily to make sure that there were no side effects, then the long-acting drug, 30 mg given every fourth week. There was a subjective improvement after the first week, and even though he still has pleural effusions bilaterally, he no longer needs palliative thoracocenteses and can live a normal life. His nails are better, as is the oedema. He is satisfied with his treatment and does not wish to have any pleurodesis. The observation time is now 6 months, and no adverse side effects have been seen so far.

Octreotide can be tried in cases of YNS before more aggressive therapies are started. However, the best results are probably achieved in the rare cases where the effusion is a chylothorax. The other symptoms, such as yellow nails and oedema, also seemed to improve but evaluation is difficult because even normally, there are variations over time with these symptoms. Pleurodesis will probably be necessary in the future for our patient despite his octreotide treatment. Further studies are warranted in this rare disease.