Abstract
Dyskeratosis congenita is a rare genodermatosis, which is characterized by triad of skin pigmentation, nail dystrophy and leukoplakic lesion in the oral cavity. The purpose of reporting this case with review of recent literature is to create better awareness about the multisystem manifestations of this fatal condition that can aid clinicians in early diagnosis. A case of 12 year old female is reported presenting with classical triad of lesions with briefly review of literature.
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