The New England Journal of MedicineYellow Nail Syndrome

Lymphatic Dysfunction Underlies Yellow Nail Syndrome, Linking Dermatological, Respiratory, and Circulatory Symptoms

Yellow nail syndrome is a rare disorder characterized by a triad of symptoms: yellow, thickened nails (xanthonychia), lymphedema, and respiratory issues such as pleural effusions or chronic sinusitis. This case report of a 67-year-old woman illustrates the classic presentation and diagnostic challenges of this condition. The syndrome’s hallmark features, although distinct, may not always occur simultaneously, complicating the diagnostic process. Current understanding points to lymphatic system dysfunction as the underlying cause, though the exact pathogenesis remains elusive.

Key Points:

• Yellow nail syndrome is characterized by xanthonychia, lymphedema, and respiratory issues
• Symptoms may occur asynchronously, making diagnosis challenging
• The condition is thought to be a disorder of the lymphatic system
• Diagnosis was made in a 67-year-old woman with chronic rhinosinusitis
• Patient presented with dyspnea, cough, yellow and thickened fingernails, and leg edema
• Chest radiograph revealed a left-sided pleural effusion
• Thoracentesis yielded 1.1 liters of milky, yellow fluid consistent with chylous effusion
• Patients with suspected yellow nail syndrome should undergo pulmonary and lymphatic evaluation
• Lymphoscintigraphy may be utilized in the diagnostic process
• Treatment is primarily supportive
• The patient received long-term treatment with pleural catheters for persistent chylous effusions
• Management involves an interdisciplinary team approach

HCN Medical Memo
Yellow nail syndrome, while rare, exemplifies the interconnectedness of dermatological, respiratory, and circulatory systems. Clinicians should maintain a high index of suspicion when encountering patients with unexplained combinations of nail changes, lymphedema, and respiratory symptoms. Early recognition and interdisciplinary management are crucial for optimal patient care.

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