|Year : 2007 | Volume
| Issue : 2 | Page : 66-68
Yellow nail syndrome
Ramakant Dixit, Kalpana Dixit, Savita Jindal
Department of Chest Diseases & Tuberculosis, B.J. Medical College & Civil Hospital, Asarwa, Ahmedabad - 380016., India
'Sita Ram Bhawan', 381/26, Ramganj, Ajmer-305001.
Source of Support: None, Conflict of Interest: None
| Abstract|| |
A case of yellow nail syndrome is described in a forty year old male patient who presented with classical triad of this syndrome i.e. deformed yellow nails, lymphedema and chronic recurrent pleural effusion. The practical problems in the diagnosis are also briefly discussed with emphasis on awareness of this rare clinical entity.
Keywords: Yellow nail syndrome, pleural effusion, lymphedema
|How to cite this article:|
Dixit R, Dixit K, Jindal S. Yellow nail syndrome. Lung India 2007;24:66-8
| Introduction|| |
The Yellow Nail Syndrome (YNS) consists of the triad of deformed yellow nails, lymphedema and pleural effusion  . The term YNS was first described by Samman and White  , for association of yellow nails with primary lymphedema in 1964, pleural effusion was later added as a frequent feature of the disease  . This is an infrequently reported clinical entity with only few cases reported from our country ,, . The present report describes a case of YNS that had all the classical features of this syndrome.
| Case Report|| |
A 40-year-old farmer presented with cough and breathlessness off & on for the past two years. He was apparently asymptomatic two years back when he first developed chest pain and shortness of breath. He was diagnosed to have left sided pleural effusion, investigated and treated as tuberculous pleural effusion (Category III in RNTCP) at that time. He completed the treatment and remained relatively asymptomatic for next six months and became symptomatic again with recurrence of pleural effusion on the same side as well as new pleural effusion on right side. He was given re-treatment regimen of tuberculosis (Category II in RNTCP) which he completed one month back. There was not much symptomatic relief during above treatment. He was non-diabetic, nonhypertensive and smoker.
General physical examination was unremarkable and body mass index was 23.4. The nails of both upper limb & lower limb were thickened, curved & yellowish brown in colour [Figure 1]. The lower limb examination revealed deformed feet with bilateral pedal edema [Figure 2]. On detailed history, the edema feet was present since last 8-10 years following a minor injury and was stationary. Examination of respiratory system revealed dull percussion note and reduced breathsounds over both lower lung fields. Other body systems were normal on clinical examination.
Routine investigations of blood and urine revealed no abnormality. X-ray chest PA view showed bilateral pleural effusion more on right side [Figure 3]. Sputum was negative for AFB and found sterile on culture. Mantoux test was negative. Other tests like serum for HIV, HBsAg, X-ray paranasal sinus and other organ function tests were also normal. Pleurocentesis was done and straw coloured clear fluid was aspirated (1000 ml from right side and 250 ml from left side) with a protein content of 3.8gm%, sugar 40 mg% & cells 250/mm3 (polymorphs 46%, lymphocytes 52%). Fluid was negative for AFB, pyogenic organisms and fungi. Pleural biopsy revealed fibrocollagenous tissue with few chronic inflammatory cells. Nails scrapings for fungus on KOH mount was also negative.
Pleural effusion on right side recurred in spite of repeated aspirations though it never became massive. Tetracycline pleurodesis was done after closed tube thoracostomy, which resulted in significant reduction in amount of free fluid in right pleural cavity.
In view of recurrence of pleural effusion despite repeated aspiration, two courses of anti tuberculosis therapy and negative work-up for any specific disease, a diagnosis of YNS was made due to associated yellow nails and lymphedema.
| Discussion|| |
Pleural effusion, although a common clinical entity in clinical practice, poses a diagnostic challenge when of chronic or recurrent nature. YNS is rare cause of such presentation with associated yellow nails and lymphedema. Some physicians restrict the diagnosis to patients who have the complete triad, whereas others accept any two characteristics  .
The three separate entities may be manifested at widely varying times  . Occasionally this syndrome may have associated bronchiectasis, sinusitis, pericardial effusion or even chylous ascites  .
In one review of 97 patients, lymphedema was first identified in neonates or in patients as old as 65 years, the mean age at onset was 40 years. Eighty-nine percent of the patients had yellow nails, 80% had lymphedema of varying severity and 36% had pleural effusion  . The basic abnormality in this syndrome appears to be hypoplasia of the lymphatic vessels which is responsible for lymphedema, nail change and pleural effusion  .
The nails are typically yellowish green in colour, thickened and excessively curved from side to side. They grow slowly and onycholysis is frequently present. The lymphedema is often mild, usually affects the lower extremities and can be demonstrated by peripheral lymphangiography , . The pleural effusions are bilateral in about 50% and vary in size from small to massive. Pleural fluid is usually clear exudate with lymphocyte predominance. Once, a pleural effusion has developed with this syndrome, it tends to persist and recur rapidly after thoracocentesis  . If the effusions are persistent and the underlying lung can be re-expanded, chemical pleurodesis or pleural abrasion can be done to prevent re-accumulation of fluid. If the lung fails to re-expand than drainage of the pleural space can be achieved by pleuro-peritoneal shunts. A recent report demonstrated the long-term efficacy of such shunts in providing palliation of symptoms  .
The syndrome has been reported in association with a variety of other diseases including immunologic deficiency in siblings thyroid disease, hypogammaglobulinemia in various forms, the nephrotic syndrome, a transient decrease in circulating B lymphocytes and protein losing enteropathy  . It is also frequently reported in association with recurrent erysipelas ,,,,,, . Hurwitz and Pinals  reported an association of chronic hereditary lymphedema with pleural effusion in two siblings and called it "Nonne Milroy Meige's disease." These cases were followed up by Siegelman and associates  , who believed that both patients had an immunologic deficiency state and demonstrated leukopenia, lymphopenia with reduction of P -globulin in the male sibling.
The diagnosis is made when a patient has a chronic pleural effusion in conjunction with yellow nails or lymphedema. The case presented here showed all the features of the classical triad of YNS. The diagnosis may be difficult or missed at times as patient may not present with all features of this syndrome simultaneously or when present with each aspect of the syndrome in the different departments. Further more, the nail changes in Indian patients may not be easily appreciable because of colour of skin. Lymphedema and chronic pleural effusion can easily be attributed to filariasis and tuberculosis respectively as both these diseases are endemic in our country. Increasing awareness of this syndrome and close scrutiny of the nails in patients having idiopathic or recurrent pleural effusion and/or lymphedema of legs will avoid diagnostic delay and other unnecessary treatment modalities.
| References|| |
|1.||Light RW. Pleural effusion. In: Murray JF, Nadel JA, editors. Text book of Respiratory Medicine. 2nd ed. Philadelphia: WB Saunders: 1994. p.2164-92. |
|2.||Samman PD, White WF. The yellow nail syndrome. Br J Dermatol 1964; 76: 153-57. |
|3.||Emerson PA. Yellow nails, lymphedema and pleural effusion. Thorax 1966; 21: 247-53. |
|4.||Ram Prasad T. Yellow nail syndrome. Chest 1980; 27: 4-6. |
|5.||Rama Prasad T. Yellow nail syndrome. Indian J Chest Dis Allied Sci 1980;22: 69-72. |
|6.||Nair VK, Sukumaran P. Yellow nail syndrome: A rarity in Indian? Indian J Chest Dis Allied Sci 1996; 38: 39-43. |
|7.||Beer DJ, Pereira W Jr. Snider GL. Pleural effusion associated with primary lymphedema : a perspective on the yellow nail syndrome. Am Rev Respir Dis 1978; 117: 595-99. |
|8.||Light RW. Pleural Diseases, Philadelphia: Lippincott Williams & Wilkins, 2001. |
|9.||Nordkild P, Kormann - Andersen H, Struve-Christensen E. Yellow nail syndrome. The triad of yellow nails, lymphedema and pleural effusions. Acta Med Scand 1986; 219: 221-27. |
|10.||Hiller E, Rosenow EC III, Olsen AM. Pulmonary manifestations of the yellow nail syndrome. Chest 1972;61 : 452-58. |
|11.||Moorjani N, Winter RJD, Yigsaw YA, Maiwand MO. Pleural effusion in yellow nail syndrome: Treatment with bilateral Pleuro peritoneal shunts. Respiration 2004; 71: 298. |
|12.||Fraser RS, Pare PD, Muller NL, Colman N. editors. Diagnosis of Diseases of the Chest. Philadelphia: WB Saunders; 1999. |
|13.||Hurwitz PA, Pinals DJ. Pleural effusion in chronic hereditary lymphedema (Nonne, Milroy, Meige's disease): report of two cases. Radiology 1964; 82: 246-48. |
|14.||Siegelman SS, Heckman BH, Hasson J. Lymphedema, pleural effusions and yellow nails: associated immunologic deficiency. Chest 1969; 56 . 114-17. |
[Figure 1], [Figure 2], [Figure 3]