Yellow nail syndrome

Published on 18/03/2015 by admin

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Last modified 22/04/2025

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Yellow nail syndrome

Robert L. Baran

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

The yellow nail syndrome (YNS) is an uncommon disorder of unknown etiology characterized by the triad of yellow nails, lymphedema, and respiratory tract involvement. This term was originally used to describe the association of slow-growing yellow nails with primary lymphedema. Pleural effusion was later recognized to be an additional sign of the syndrome. Since then, other respiratory conditions, such as bronchiectasis, sinusitis, bronchitis, and chronic respiratory infections have been associated with the disorder. Although all the three signs that classically characterize the triad of YNS do not occur in every patient, the presence of typical nail alterations should be considered an absolute requirement for the diagnosis. The complete triad is seen in 25% of patients, lymphedema in 40%, and pleural effusions in only 2% of patients with yellow nails. A variant of yellow nails can also be seen in HIV infection.

Management Strategy

Although YNS may resolve spontaneously, treatment is often sought by sufferers. The yellow nails are unsightly, discolored, hard, show transverse over-curvature, and are slow growing. Paronychia and onycholysis can be observed.

Underlying diseases such as respiratory disorders, malignancy, infections, immunologic and hematologic abnormalities, endocrine, connective tissue and renal abnormalities, and miscellaneous disorders, including penicillamine therapy, should be sought. Titanium or titanium dioxide may cause YNS. Improvement of any underlying disorder (e.g., lymphedema) may also result in improvement of the nail plate.

There are no large series or randomized trials in the treatment of YNS. Oral vitamin E has been used as monotherapy. The oral azole antifungals itraconazole and fluconazole have also been reported to be effective, both alone or in combination with vitamin E. The combination of fluconazole and vitamin E is currently the best treatment for curing the nail unit. This was confirmed by anecdotal reports that supplement our own findings. Matrix steroid injections have been successfully employed. Octreotide, zinc, and medium chain fatty acid triglyceride supplements as well as clarithromycin have been used in anecdotal reports.

Attention to the diseases underlying yellow nails is mandatory but does not always result in resolution of the nail dystrophy. Therapies that effect resolution of the nail changes in yellow nails may have no effect on the associated pathology.

First-line therapies

image α-Tocopherol + fluconazole B
image Treatment of the concomitant disorder E
image Stopping galvanic release of titanium ions. B

Second-line therapies

image Intradermal triamcinolone injections to the proximal nail matrix B
image Clarithromycin E
image Physiotherapy E

Third-line therapy

image Oral zinc supplementation E
image Dietary treatment E
image Octreotide treatment E

[/level-membership-for-dermatology-category][not-level-membership-for-dermatology-category]

Yellow nail syndrome

Robert L. Baran

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

The yellow nail syndrome (YNS) is an uncommon disorder of unknown etiology characterized by the triad of yellow nails, lymphedema, and respiratory tract involvement. This term was originally used to describe the association of slow-growing yellow nails with primary lymphedema. Pleural effusion was later recognized to be an additional sign of the syndrome. Since then, other respiratory conditions, such as bronchiectasis, sinusitis, bronchitis, and chronic respiratory infections have been associated with the disorder. Although all the three signs that classically characterize the triad of YNS do not occur in every patient, the presence of typical nail alterations should be considered an absolute requirement for the diagnosis. The complete triad is seen in 25% of patients, lymphedema in 40%, and pleural effusions in only 2% of patients with yellow nails. A variant of yellow nails can also be seen in HIV infection.

Management Strategy

Although YNS may resolve spontaneously, treatment is often sought by sufferers. The yellow nails are unsightly, discolored, hard, show transverse over-curvature, and are slow growing. Paronychia and onycholysis can be observed.

Underlying diseases such as respiratory disorders, malignancy, infections, immunologic and hematologic abnormalities, endocrine, connective tissue and renal abnormalities, and miscellaneous disorders, including penicillamine therapy, should be sought. Titanium or titanium dioxide may cause YNS. Improvement of any underlying disorder (e.g., lymphedema) may also result in improvement of the nail plate.

There are no large series or randomized trials in the treatment of YNS. Oral vitamin E has been used as monotherapy. The oral azole antifungals itraconazole and fluconazole have also been reported to be effective, both alone or in combination with vitamin E. The combination of fluconazole and vitamin E is currently the best treatment for curing the nail unit. This was confirmed by anecdotal reports that supplement our own findings. Matrix steroid injections have been successfully employed. Octreotide, zinc, and medium chain fatty acid triglyceride supplements as well as clarithromycin have been used in anecdotal reports.

Attention to the diseases underlying yellow nails is mandatory but does not always result in resolution of the nail dystrophy. Therapies that effect resolution of the nail changes in yellow nails may have no effect on the associated pathology.