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Neil Lagali, PhD; Anette Dellby, RN; Per Fagerholm, MD, PhD

Arch Ophthalmol. 2009;127(6):816-818.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Darier-White disease (also known as Darier disease or keratosis follicularis spinulosa decalvans) is a rare dominantly inherited skin disorder characterized by firm, scaly, cutaneous papules and plaques distributed over various regions of the body.¹ Histopathologic and electron microscopic studies of biopsied skin specimens have revealed a loss of cell to cell adhesion and abnormal differentiation of the epidermis.¹ Ocular involvement in Darier-White disease has been observed, with eyelid and corneal abnormalities being reported.^2-5 To date, however, examination of corneal abnormalities in Darier-White disease has been limited to slitlamp observation^2-5 and microscopic examination of superficial peripheral biopsy samples in cases with confirmed corneal abnormalities.^2-3

In this article we use in vivo confocal microscopy to describe the general corneal morphological features present in 5 members (4 affected and 1 unaffected) of a 5-generation, 32-member, Swedish family with Darier-White disease, 15 of whom . . . [Full Text of this Article]

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