Diagnosis and management of yellow nail syndrome: An international multi-institutional retrospective cohort study of 111 cases by an expert panel.

Abstract

Yellow nail syndrome (YNS) is a rare syndrome with unknown pathogenesis characterized by a clinical triad of respiratory disease, lymphedema, and xanthonychia. Diagnosis is challenging as clinical manifestations often do not occur concurrently. Due to disease rarity, few studies to date have characterized YNS or assessed therapeutics. We aimed to characterize clinical findings of YNS and compare therapeutic regimens to provide diagnostic and management recommendations. An international multi-institutional retrospective cohort study of YNS patients was conducted by a panel of nail experts. We included 111 YNS patients. Xanthonychia, increased transverse curvature, thickening, nail growth arrest, and a history of respiratory disease or lymphedema were highly suggestive of YNS. The therapeutic regimen with highest complete response rate was oral vitamin E combined with oral azole antifungals. Limitations include nonrandomized and retrospective design. Clinical details were inconsistently reported. Some treatments discussed are expert opinions and based on hypothetical and/or minimal data. We provide the largest study to date characterizing YNS. We suggest that patients with suspected YNS undergo mycologic examination and referral to pulmonology. We recommend oral vitamin E with oral azole antifungal as the first-line therapy for YNS. Reassurance/no treatment could also be considered.