Yellow nail syndrome

The yellow nail syndrome was first described in 1964 [1] with 13 cases of lymphedema associated with nail dystrophy. Pleural effusions, bronchiectasis, and sinusitis have also been reported. [2] Hard wax impaction in the external auditory meatus, thought to represent keratosis obturans, has been associated with the yellow nail syndrome in one case report. [3] We describe a patient in whom all these features and keratosis obturans coexist.

CASE REPORT

A 40-year-old woman first presented in 1955 with nasal blockage and sinusitis. At the age of 55 years, a diagnosis of the yellow nail syndrome was made on the basis of chronic nail changes and persistent mild edema of her face and ankles.

At age 64 years , she was referred to the Osler Chest Unit, Oxford, England, because of a two-year history of productive cough and recurrent chest infections. A clinical diagnosis of bronchiectasis was made. She had gross nail deformity with yellow discoloration, moderate facial swelling, minimal ankle edema, but no pleural effusions. Spirometry showed [FEV.sub.1] of 1.7 and FVC of 2.7 L (BTPS). She had been a regular heavy smoker.

Bilateral conductive deafness led to a diagnosis of keratosis obturans of the external auditory meatus. This has been removed at regular intervals since with hearing improvement each time.

In 1981, when she was 66 years old, bilateral pleural effusions were noted. These were tapped but they reaccumulated rapidly. Cytologic study of the pleural fluid showed no malignant cells, culture was negative, and the protein content was 5.4 g/dl. Other investigations were normal except for a raised erythrocyte sedimentation rate (ESR) at 69 mm/h. Spirometry in the presence of the effusions gave an [FEV.sub.1] of 1.02 L and FVC of 1.69 L (57 percent and 66 percent predicted respectively). Because of persistent dyspnea due to recurrent pleural effusions, she underwent successful tetracycline pleurodeses. Her condition remains stable three years later with bilateral basal pleural thickening but no obvious effusions. Most recent spirometry in 1989 gave an [FEV.sub.1] of 1.5 L and FVC of 2.5 L (predicted, 1.7 L and 2.4 L).

DISCUSSION

The yellow nail syndrome has now been well characterized and consists of yellow nails, lymphedema, and pleural effusions. Bronchiectasis and sinusitis are often associated.

The etiology is unknown, but lymphagiography [1] has shown very variable lymphatic hypoplasia. Fluid kinetics of the pleural effusions suggest that the accumulation is due to ineffective drainage rather than excess production of pleural fluid. [4] Pleural effusions often occur late in life and are sometimes preceded by infection.

Moran and Larkworthy [3] speculated that keratosis obturans was a manifestation of the yellow nail syndrome in the external auditory meatus. However, in an early description of the association between bronchiectasis and keratosis obturans, [5] a secretomotor reflex mediated through vagal channels in the auditory branch of the vagal nerve was postulated. An active cough reflex induced by stimulation of that nerve was cited as evidence; also, the bronchiectasis and the ear problems were usually ipsilateral. In patients without associated bronchiectasis, an underlying hyperemia and chronic inflammation have been observed in the external auditory meatus with keratosis obturans and consequent increased desquamation suggested as the mechanism for the keratin plug.

If keratosis obturans is a manifestation of the yellow nail syndrome, our patient represents the first report in which all the different features coexist. It is now 20 years since the diagnosis was made and she remains well, emphasizing the good prognosis in these patients.

REFERENCES

[1] Samman PD, White WF. The yellow nail syndrome. Br J Dermatol 1964; 76: 153

[2] Hiller MD, Rosenow EC, Olsen AM. Pulmonary manifestations of the yellow nail syndrome. Chest 1972; 61: 452-58

[3] Moran MF, Larkworthy W. Upper respiratory problems in the yellow nail syndrome. Clin Otolaryngol 1976; 1: 333-36

[4] Runyon RA, Forker EL, Sopko JA. Pleural-fluid kinetics in a patient with lymphedema, pleural effusions and yellow nails. Am Rev Respir Dis 1979; 119: 821-25

[5] Morrison AW. Keratosis obturans. J Laryngol Otol 1956; 70: 317-21

COPYRIGHT 1990 American College of Chest Physicians
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