Xeroderma pigmentosum (XP) is a rare genetic disease characterised by defective DNA repair leading to clinical and cellular hypersensitivity to ultraviolet radiation. The oculocutaneous features of 10 patients with XP were studied retrospectively. General features included parental consanguinity (40%), familiality (60%), onset of symptoms in first 2 years (50%), malignant skin neoplasms (60%), and carcinoma of the tongue (20%). Among the ocular features, 50% ofpatients presented with photophobia. Lid freckles or atrophic skin lesions were seen in all patients. Lower lid tumours were seen in 30%, chronic conjunctival congestion in 40%, corneal opacification in 40%, squamous celi carcinoma of limbus in 20%, bilateral pterygium in 40%, and visual impairment in 50%. The clinical features Departments of (ocular and cutaneous) of the cases are disOphthalmology and cussed.
Corneal collagen CXL was found to be safe and effective in the first 12 months for keratoconus in children with similar initial efficacy as in adults in terms of improvement in visual and topographic outcomes.
Topical 1% voriconazole was found to be safe and effective drug in primary management of fungal keratitis, its efficacy matching conventional natamycin. There was no added advantage of using topical 1% voriconazole over topical natamycin as primary treatment in fungal keratitis.
Corneal CXL is more effective in improving the refractive and topographical parameters at 1 year when it is performed early in the course of the disease.
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