Abstract:Darier's disease is an autosomal dominant disorder characterized clinically by presence of keratotic papules in a seborrheic distribution, nail involvement and mucosal lesions. There are several clinical variants of Darier's disease, but few cases of segmental Darier's disease have been described in the literature. We describe a 12year old boy with type 2 mosaic phenotype. This unique clinical variant of Darier's disease has been described very rarely.
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