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Amyloid diseases are secondary protein structure diseases in which insoluble protein fibrils accumulate extracellularly. Twenty different types of fibrils have been described in human amyloidosis, each with a different clinical picture. Amyloidosis can be generalised, affecting multiple organ systems, or localised and can affect almost any organ of the body. In the eye amyloid is the material commonly seen in lattice corneal dystrophy. Cases of localised corneal amyloidosis have been reported in literature but are quite rare.1–4 We report a case of localised corneal amyloidosis presenting as a large raised gelatinous vascularised lesion in a patient with long standing herpetic keratitis.
A fit and healthy 34 year old woman was a tertiary referral to the corneal clinic with a long standing history of a lesion on her right cornea. The initial presentation as a teenager was of a red sore right eye with a corneal ulcer that was treated as a bacterial ulcer for a few years and later on as recurrent herpetic keratouveitis. She had had numerous intermittent courses of combined topical antivirals and steroids with resolution of symptoms. Over the past 2 years she was noted to develop a raised vascularised lesion over the right cornea, which gave a constant foreign body sensation and occasional episodes of pain. It was the appearance of the lesion and the discomfort rather than the reduced visual acuity, which prompted her to seek treatment. On presentation in the clinic she had a visual acuity of 6/36 right (6/24 with pinhole) and 6/6 left eye. Anterior segment examination showed a large, raised, gelatinous, slightly nodular, vascularised lesion on the right cornea (Fig 1). The rest of the anterior segment examination was normal. Ocular adnexae did not show any signs of chronic lid disease. The corneal sensation was intact. A superficial keratectomy was performed under general anaesthesia to excise the lesion. Histopathological examination of the specimen revealed a diagnosis of amyloidosis (Fig 2).
Amyloidosis can be either primary or secondary, both of which can be further classified into systemic and local disease. Systemic primary amyloidosis can affect various ocular structures presenting as papules or purpura on the lids, conjunctival deposits, external ophthalmoplegia, vitreous opacities, and glaucoma. Secondary systemic amyloidosis rarely affects the eye, although a case of conjunctival amyloidosis has been reported in a patient with rheumatoid arthritis.5
Stafford and Fine, for the first time in 1966, reported a case of corneal amyloidosis in a young girl with ocular complications of retinopathy of prematurity.6 Primary familial amyloidosis, which presents as nodular white subepithelial protuberances in the central cornea, has been postulated to be autosomal recessive.1 In secondary localised corneal amyloidosis, the material is deposited as a result of chronic inflammation and irritation from scarred lids from trachoma, trichiasis, or long standing corneal scars.2–4,7–9 To the best of our knowledge its association with herpetic keratitis has not been reported.
Macpherson et al retrospectively examined 200 specimens of corneas removed for various reasons specifically for amyloid deposits and found it present in seven cases (3.5%).7 It has been proposed that the basal cells of the corneal epithelium are responsible for the synthesis of amyloid,2 although other sources have been also proposed.8
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