Yellow nail syndrome (YNS) is a very rare syndromic disorder characterized by the variable triad of characteristic yellow nails, chronic respiratory manifestations, and primary lymphedema .
Summary
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- 1 Epidemiology
- 2 Clinical description
- 3 Etiology
- 4 Diagnostic methods
- 1 Differential diagnosis
- 5 Management and treatment
- 6 Forecast
- 7 Sources
epidemiology
Prevalence and incidence rates are unknown, but YNS is considered a very rare disease . More than 150 cases have been described to date. The disease equally affects men and women.
Clinical description
The clinical signs of YNS usually develop after puberty, but there is wide variability in the age of onset. Most cases are late onset, after age 50. Patients generally complain of slowing down or stopping nail growth: the nails become thicker and more opaque, presenting a yellowish or greenish coloration and without cuticles. Increased transverse curvature is another feature, which can lead to detachment of the nail plate. Nail changes tend to affect all 20 nails, but the severity is variable. The lymphedema is the most consistent finding usually affect the lower limbs. YNS may be associated with respiratory involvement with coughchronic, bronchitis , tracheobronchitis, bronchiectasis , chronic sinusitis , and pleural effusions. Some patients have all three signs of the triad, while others have the nail changes with lymphedema or respiratory disorders, or just nail changes. YNS can also be a paraneoplastic disorder.
Etiology
The etiology of YNS has not been elucidated, although it is believed to be caused by an underlying lymphatic abnormality, with reduced lymphatic drainage.
Diagnostic methods
Diagnosis is usually based on the presence of two of the three classic clinical manifestations, including nail changes, respiratory disorders, and lymphedema , or on highly characteristic nail changes. This is because the three main signs may not be present at one time in the patient. Diagnosis can also be based only on typical nail changes.
Differential diagnosis
The most important differential nail diagnosis includes chronic paronychia , with which it shares the absence of a cuticle , the thickening and coloring of the nail , and onychomycosis of the toenails, which produces thickening and onycholysis. Onychogryphosis and acquired pachyonychia should also be considered .
Management and treatment
Spontaneous improvement in nail signs has been reported in more than 30% of cases. The daily dose of 1,200 IU of systemic vitamin E has also been described to be effective in more than half of patients.
Forecast
Some patients make a full recovery, but the respiratory disease and associated symptoms are often chronic. The Lymphedema severe respiratory complications and can affect the quality of life of those affected and is forced to follow regularly.
