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Darier-White disease, also known as keratosis follicularis, is a relatively common genodermatosis (frequency between 1 in 36 000 and 1 in 100 000) with autosomal dominant inheritance and late age of onset.1 Clinical features consist of disseminated warty papules and plaques with an affinity to the seborrhoeic areas of the body. The disease may be exacerbated by sun exposure or sweating and never remits.
Ocular involvement in keratosis follicularis is relatively uncommon. Most of the affected patients have dry eye syndrome with and without Sjögren’s syndrome. Possible corneal manifestations are peripheral arcus lipoides-like opacifications, asymptomatic nebular dot-like opacities of the peripheral corneal epithelium, and central epithelial surface irregularities.2–4
Involvement of the eyelids and association with retinitis pigmentosa, subnormal dark adaption, and post-cataract extraction staphylococcal endophthalmitis may also occur.4,5
We describe the first case of recurrent severe bilateral corneal ulcerations with perforation in a female patient with keratosis follicularis.
A 74 year old woman was referred to the university eye hospital with complaints of redness and reduced visual acuity in the left eye. She had no significant ocular history. …
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