Dariers disease (Keratosis follicularis) is an autosomal dominantly inherited, acantholytic disorder due to mutation in ATP2A2 gene located on chromosome 12. Darriers disease can be localised to a segment due to postzygotic mutations where it is called as segmental darriers. Clinical features of DD include greasy, warty papules and plaques on seborrheic areas, dystrophic nails, palmo-plantar pits and papules on the dorsum of the hands and feet. There is increased predisposition to non-melanoma

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... utaneous malignancies in darriers disease. We report a rare case of darriers disease with basal cell carcinoma A, with rapid progression over 4 months into ulcus penetrans with destruction of eye ball.