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Rare disease
Dyskeratosis congenita with malignant transformation
  1. Jay Gopal Ray,
  2. Niharika Swain,
  3. Ranjan Ghosh,
  4. Richa,
  5. Sweta Pattanayak (Mohanty)
  1. Department of Oral and Maxillofacial Pathology, Dr R Ahmed Dental College & Hospital, Kolkata, India
  1. Correspondence to Jay Gopal Ray, jaygopalray{at}


Dyskeratosis congenita (DC) is a rare genodermatosis characterised by a classic triad of dystrophic nails, reticular skin pigmentation and mucous membrane leukoplakic patches, which have a high rate of malignant transformation. The case report presented here deals with a sporadic case of DC without similar clinical presentation in the first-degree and second-degree relatives. Of note in this case, there was rapid malignant transformation in the non-homogeneous nodulo-speckled leukoplakic patch on the dorsum of the tongue.

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  • Competing interests None.

  • Patient consent Obtained.