Yellow Nails, Lymphedema and Pleural Effusion
The triad of yellow nails, lymphedema and pleural effusion was first described by Emerson in 1966 and was labelled the yellow nail syndrome. Since then, more than 100 patients with the classic triad or variants thereof have been described. Pleural effusions in this syndrome range from small, unilateral and asymptomatic to large, bilateral and debilitating. We describe a patient with yellow пай syndrome and symptomatic chronic pleural effusion inadequately controlled with frequent thoracentesis but successfully treated with pleuroperitoneal shunting.
A 65-year-old white woman was referred to the Pulmonary Clinic of the University of Chicago Hospitals and Clinics in November 1986 for evaluation of a left pleural efiusion that was found on routine physical examination. The patient admitted to a several month history of mild fatigue and dyspnea upon walking several blocks. She noted pretibial and ankle edema bilaterally for several years, which was stable, and did not respond to leg elevation, support stockings or a trial of diuretics. She stated that since 1980, her nails had been thick, yellow and would break easily, and she always wore nail polish. She denied cough, fevers or night sweats. She had never smoked and had no known occupational exposure. There was no family history of lymphedema, bronchiectasis, sinusitis or immune deficiency.
FIGURE 1. Patient s fingers. Note yellow color, thickening, transverse ridging, onycholysis and absence of lunulae.
Physical examination was remarkable for dullness and decreased breath sounds at the left lung base. There was 1+ pretibial and ankle edema, which pitted slightly and was not brawny. The nails were yellow, thickened, onycholytic and had transverse ridging. The lunulae were absent (Fig 1).
Chest radiograph revealed a large left pleural effusion with a normal heart and pulmonary vasculature (Fig 2). A 5-TU PPD was read as positive with 10 mm of induration. An echocardiogram was normal. Thoracentesis revealed straw-colored fluid with 150 red blood cells/jtL; 2,800 white blood cells/|iL; 93 percent lymphocytes; 5 percent macrophages; 1 percent eosinophils; 1 percent mesothelial cells. The protein was 5.5 g/d\; LDH, 150 IU/L; pH 7.44. The AFB smear and culture were negative. Pleural biopsy revealed chronic inflammation without granulomata or giant cells. The AFB stain and culture of the biopsy specimen were negative. Fungal cultures of nail clippings were negative.
FIGIURE 2. Chest radiograph at time of presentation, demonstrating left pleural effusion and otherwise normal lung fields.
A diagnosis of pleural effusion secondary to yellow nail syndrome was made. Over the following eight months, the patient required thoracentesis and removal of 1,500 ml of pleural fluid every four weeks for relief of severe fatigue, dyspnea on exertion and orthopnea. The symptom-free interval between taps was approximately three weeks. Pleural fluid analysis of all subsequent therapeutic taps was similar to the initial values. The AFB cultures and cytologic findings on all specimens were negative. In March 1987, a small right pleural efiusion was noted on chest radiograph.
Because of declining efficacy of thoracentesis in relieving her symptoms, obliteration of the pleural space was considered but the patient refused both chemical pleurodesis and open pleural abrasion or pleurectomy. In July 1987, the patient underwent placement of a left pleuroperitoneal shunt (Denver Biomaterial Inc, Denver) with a 1.5 ml pumping chamber. Thoracoscopy performed at that time was unremarkable. The patient was instructed to compress the pumping chamber 100 cycles four times per day. Despite good compliance with this regimen, three weeks after placement of the shunt the patient was severely fatigued and dvspneic, and chest radiograph showed reaccumulation of the left pleural effusion. It was assumed that the shunt had become obstructed.
FIGURE 3. Chest radiograph one month after placement of successful pleuroperitoneal shunt. Shunt is visible in left lower lung field. Note marked decrease in left pleural effusion and new right pleural effusion.
The patient underwent thoracentesis every four weeks until November 1987, when she underwent placement of a second pleuroperitoneal shunt. The patient was again instructed to compress the pumping chamber 100 cycles four times per day. Since the placement of this second shunt the patient has been followed up for one year and has not required thoracentesis, nor has she had any recurrence of her symptoms. Chest radiographs have shown a stable left pleural effusion, decreased in size compared with preoperative films, and a small, stable right pleural effusion (Fig 3).