Yellow Nail Syndrome


Yellow Nail Syndrome




Training Level

Resident PGY 2


Henry Ford Hospital


HISTORY: A 62-year-old Caucasian female presented for finger and toenail yellowing with associated poor nail growth and intermittent nail shedding over the past 10 years. She also has a history of recurrent pulmonary effusions and lower extremity lymphedema that was diagnosed at approximately the same time. EXAMINATION: There was yellow discoloration of all fingernails and toenails with prominent distal onycholysis without subungual debris. Lunula and cuticles were absent. Fingernails were more affected than toenails. Some nails had slightly thickened and overly curved nail plates without other dystrophic changes. The bilateral lower legs (left worse than right) demonstrated lymphedema. COURSE AND THERAPY: Nail PAS and fungal culture were negative. Prior to presentation, patient received courses of oral terbinafine and fluconazole without improvement. She was placed on vitamin E 1000 units daily and pulse fluconazole for 3 months. Due to minimal improvement, fluconazole was stopped; however, vitamin E was continued. She also performs dilute vinegar soaks to reduce potential superinfection in the setting of onycholysis. Recently, she underwent pulmonary wedge resection and right partial pleurectomy. With regards to her lymphedema, she has been using home pneumatic compression pumps with transient relief. DISCUSSION: Yellow nail syndrome (YNS) is a rare idiopathic disorder characterized by the triad of yellow nails, lymphedema, and respiratory tract problems, however this classic triad occurs only in about one-third of patients. The diagnosis only requires the presence of the typical nail changes. Lymphedema and respiratory tract involvement may develop before, during, or after the nail dystrophy. YNS affects men and women equally, and typically presents in the fourth to sixth decade of life.All nails may be affected, and nails are usually slow growing or appear to have stopped growing. Nails become thicker and turn a pale yellow or green-yellow color with slightly darker edges. Nails can remain smooth, develop cross-ridging, or prominent curvature transversely and longitudinally. There is a loss of cuticles, and the lunula is no longer visible. Onycholysis may affect one or more nails and may extend proximally to the distal matrix, causing nail shedding.The cause of yellow nail syndrome is relatively unknown with most cases occurring sporadically. Possible etiologies include microangiopathy with protein leakage or dysfunctional lymphatic drainage. Lymphedema occurs in about 80% of patients and most frequently affects legs. Respiratory tract involvement usually manifests as pleural effusions, affecting 36% of patients. Patients can also provide a history of recurrent attacks of bronchitis, chronic sinusitis, and pneumonia. Improvement in respiratory disease has been linked to improvement in the appearance and growth of nails. Vitamin E, recommended at 1200 IU daily, has been reported to improve the appearance of nails. Additionally, pulse therapy with itraconazole or fluconazole has been reported to stimulate nail growth and may be given in combination with vitamin E.

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Yellow Nail Syndrome