Background: Yellow nail syndrome (YNS) is a rare condition defined by the presence of yellow nails associated with lymphedema and/or chronic respiratory manifestations. Several aspects of this disorder remain poorly defined.
Methods: We sought to clarify the clinical features and course associated with YNS by analyzing 41 consecutive cases evaluated at a tertiary referral medical center.
Results: There were 20 men and 21 women; median age at diagnosis was 61 years (range, 18 to 82 years). None had a family history of YNS. All but one patient had chronic respiratory manifestations that included pleural effusions (46%), bronchiectasis (44%), chronic sinusitis (41%), and recurrent pneumonias (22%); 26 patients (63%) had lymphedema. Treatment included rotating antibiotic therapy for bronchiectasis, thoracenteses, oral vitamin E, and corticosteroid therapy. Eight patients underwent surgical management of recurrent pleural effusions including pleurodesis and decortication; two additional patients underwent pleurodesis via tube thoracostomy. The yellow nails improved or resolved in 14 of 25 patients (56%) for whom relevant data were available. Median survival of this cohort using the Kaplan-Meier method was 132 months, significantly lower than (p = 0.01) the control population. Among those still alive (20 patients), the disease appeared stable.
Conclusions: In most cases, YNS is an acquired disorder and associated respiratory manifestations are generally manageable with a regimen of medical and surgical treatments. Yellow nails improve in about one half of patients, often without specific therapy.