Abstract

BackgroundYellow nail syndrome (YNS) is a rare disease characterized by the triad of thickened, slow-growing yellow nails, lymphedema, and chronic respiratory manifestations. The cause of YNS is not known; however, it is suggested to be due to a congenital lymph abnormality. Since YNS is accompanied by chronic bronchial infection in more than half of patients, we hypothesized that treatment with clarithromycin (CAM) could be effective. We therefore evaluated the effectiveness of CAM against nail discoloration and respiratory manifestation in patients with YNS.MethodsWe conducted an observational study involving 5 patients with YNS who were treated at our institution between January 2005 and January 2016. CAM was prescribed for every patient. Patient demographic information, comorbidities, medications, chest radiographs, and clinical data such as nail color were extracted to evaluate clinical outcome.ResultsMean patient age was 71.6 years, and 2 patients (40%) were male. Four patients had sinusitis, and 2 had rheumatoid arthritis. Regarding respiratory manifestations, 4 patients had sinobronchial syndrome and 2 had pleural effusion. Nail discoloration improved in every patient after CAM treatment. Four patients also experienced improvement in their respiratory manifestations.ConclusionsIn patients with YNS, the anti-inflammatory activity of macrolides might improve their systemic inflammation. This improvement could help to reduce lymphedema and promote nail growth.Trial registrationEthical approval was provided by the institutional review board of the National Center of Global Health and Medicine (NCGM-G-002143-00), in January 2017. This study is retrospectively registered for UMIN Clinical Trial Registry (UMIN000028514) in August 4th, 2017.

Highlights

  • Yellow nail syndrome (YNS) is a rare disease characterized by the triad of thickened, slow-growing yellow nails, lymphedema, and chronic respiratory manifestations

  • Since Emerson’s 1966 report describing respiratory complications of the disease, YNS has been defined by the classical triad of yellow nails, lymphedema, and chronic respiratory manifestations [2]

  • We conducted an observational study at the National Center of Global Health and Medicine (NCGM), a general hospital of the National Research and Development Agency in Tokyo, Japan, which has more than 700 inpatient beds

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Summary

Introduction

Yellow nail syndrome (YNS) is a rare disease characterized by the triad of thickened, slow-growing yellow nails, lymphedema, and chronic respiratory manifestations. We evaluated the effectiveness of CAM against nail discoloration and respiratory manifestation in patients with YNS. Yellow nail syndrome (YNS), a rare disease first described in 1964 by Samman and White [1], is characterized by yellow nails due to nail growth delay and lymphedema. Since Emerson’s 1966 report describing respiratory complications of the disease, YNS has been defined by the classical triad of yellow nails, lymphedema, and chronic respiratory manifestations [2]. The nail grows more slowly than 0.2 mm/week compared with the minimum of 0.5–1.2 mm/week in healthy subjects [2] These nail changes are thought to be an abnormality of growth. Defective lymphatic drainage around nails, usually congenital [1], microvasculopathy with protein leakage [4], accumulation of lipofuscin pigment [6], and titanium ion Matsubayashi et al BMC Pulmonary Medicine (2018) 18:138 involvement [7] might be considered causes of nail abnormality

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