Yellow Nails, Chronic Cough, and Edema

Presentation 

An 86-year-old man was plagued by seemingly disparate complaints. The patient had edema of the right leg and a long history of chronic cough and recurrent pneumonia. He also reported bleaching of all 20 nails, and he found that over several months, they had deteriorated, ultimately developing a yellowish hue. In addition, they seemed to be growing more slowly than was normal. He had no positive family history for any of these conditions, and no history of chronic sinusitis.

Assessment 

Physical examination revealed a straw-yellow discoloration of all finger- and toenails (Figure 1, Figure 2, Figure 3). The whole nail plate was affected. In addition, the patient had horizontal overcurvature of the nail edges, thickening of the nails, and a loss of lunulae and cuticles.

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Figure 1. Lymphedema of the patient's right leg was evident. Note the swelling at the ankle and the yellowish discoloration of the toenails.

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Figure 2. The patient's yellow thumbnail is compared with the normal thumbnail of the author (seen at left).

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Figure 3. Yellow fingernails of the patient's left hand are compared with normal fingernails of the author (seen at right).

Edema was especially pronounced below the right knee. No signs of chronic venous insufficiency, such as skin discoloration, or of trophic changes were evident. The Stemmer sign was positive, consistent with a diagnosis of lymphedema. That is, the skin at the base of the second toe could not be folded between the fingers and lifted. Chest radiography identified bilateral pleural effusions, and suspected bibasilar bronchiectasis was later confirmed with a computed tomography scan of the chest (Figure 4).

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Figure 4. A chest x-ray showed bilateral pleural effusions and bronchiectasis.

Diagnosis 

Yellow nail syndrome, first described in 1964 by Samman and White, is defined by the triad of yellow nails, lymphedema, and chronic respiratory manifestations.1 At least 2 of these 3 attributes must be present for the diagnosis, which is entirely clinical.2, 3

Possible respiratory manifestations include pleural effusions, bronchiectasis, rhinosinusitis, chronic cough, and recurrent lung infections. The finger and toenails acquire a pale yellow or somewhat greenish color, which usually involves the entire nail plate; occasionally, the proximal half of the nail might be spared. Furthermore, excessive transverse and longitudinal overcurvature, onycholysis, and shedding of the nails can occur.2, 3 The lateral edges retract so that they recede from the adjacent soft tissues.4 Published case reports of patients with yellow nail syndrome detail other clinical associations with the disorder, such as connective tissue diseases, malignancy, immunodeficiency, adverse drug reactions, diabetes mellitus, and thyroid dysfunction.2

Not surprisingly, the pathophysiology of this syndrome remains obscure. Anatomic and functional lymphatic drainage anomalies have been suggested as an underlying cause. Paraneoplastic syndrome, autoimmune disease, and genetic factors also have been mentioned as a possible basis for the disorder. So, the diagnosis has to be established by clinical signs or by exclusion.

Management 

Among therapies used to specifically treat the yellow nails are oral antifungal agents, zinc, topical or oral vitamin E, and intralesional steroid injections. Luyten et al reported improvement of the nails after administration of oral vitamin E and treatment of the respiratory pathology.5 They had even better results after introduction of itraconazole pulse therapy for secondary onychomycosis.

Tosti et al administered itraconazole, 400 mg daily for 1 week per month, over a period of 6-12 months.6 A complete cure was achieved in only 2 of 8 patients; 2 more patients had mild improvement and 4 patients had no improvement at all. Since itraconazole is quite expensive, can be difficult to tolerate, and is of questionable efficacy in yellow nail syndrome, Tosti and colleagues concluded that itraconazole should not be recommended for treatment of the disorder. Vitamin E, 1200 mg daily, is the only treatment that has been applied in a substantial number of patients, although it is not always completely successful.7, 8, 9 Spontaneous remission of nail changes has been described in up to one third of patients.

Our patient, citing advanced age, refused treatment for his disease.