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The yellow nail syndrome (YNS) is a rare clinical entity characterised by slow growing yellow discoloured nails in association with peripheral lymphoedema, pulmonary manifestation (bronchiectasis, pleural effusions), and chronic sinusitis.1 About 100 cases of YNS have been described in the literature but it has never been associated with ocular manifestations.2 We report one patient with YNS in whom chronic chemosis and conjunctival degenerative lesion was observed.
A 61 year old man was referred with a 2 month history of ocular irritation in the left eye. His past medical history was significative for yellowish slow growing nails, chronic maxillary sinusitis, and bronchiectasis for 5 years diagnosed as YNS. On examination, nails of both hands and feet showed yellow discoloration and thickening (Fig 1). He also had oedema over the lower limbs and subacute bronchial infection for 2 months. On ocular examination, corrected visual acuity was 20/20 in both eyes. Slit lamp examination of the left eye revealed an area of conjunctival injection and thickening in the palpebral fissure adjacent to nasal limbus. A mild nasal chemosis and superficial corneal micropannus were also observed (Fig 2 top). Tear secretion was normal. Examination of the anterior and posterior segment in both eyes was unremarkable. Treatment with topical corticosteroid (dexamethasone 0.1%, four times per day) and artificial tears was begun. Two weeks after presentation the patient reported an incomplete resolution of the irritation on the left eye and conjunctival lesion persisted. Computed tomographic scan of the brain and the orbits was normal. A conjunctival biopsy specimen showed multilayered epithelium, nuclear pleomorphism with malpighian differentiation. The underlying connective tissue was normal (Fig 2 bottom). Ocular symptoms improved rapidly and postoperative antibiotic and steroid eye drops where discontinued after 2 weeks. There was no recurrence of the conjunctival lesion but a mild chemosis persist in the left eye and appeared in the right eye.
YNS was first described by Samman and White in 1964.1 The syndrome has been defined as the complete triad of slow growing yellow nails, lymphoedema, and pleural effusions but today it is accepted that the presence of two of the three symptoms is sufficient to establish the diagnosis, even though the abnormalities may appear separately with intervals of several years.2
The aetiology of YNS is obscure, while pathogenesis seems to involve impaired lymphatic drainage.3 Ocular involvement has never been described in the YNS. However, conjunctival chemosis observed in our patient could be related to YNS which may involve the pleura, the lungs, but also other serosal or mucosal membranes such as the conjunctiva. Moreover, ocular symptoms and corneal micropannus appeared at the same time of a rise of pulmonary symptoms. An inflammatory component, which could alter blood flow and capillary permeability, has not been excluded and, interestingly, topical dexamethasone used to treat chemosis is efficient.
There is very little information on histopathological findings observed in the YNS. Nail biopsies demonstrated features of maturation disarray in the nail bed epithelium associated with dense, fibrous tissue replacing subungual stroma.4 The changes that we observed in the conjunctival epithelium are similar to that in the nail bed in YNS.
Since it appears that there is a pathogenic association between the YNS and ocular findings, this association is probably not coincidental and could be the first report of ocular manifestation of YNS.
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