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Dyskeratosis Congenita; A case report and review of literature

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Author(s): Latha Mary Cherian | Deepthi K | Rejani E.R

Journal: Oral & Maxillofacial Pathology Journal
ISSN 0976-1225

Volume: 1;
Issue: 2;
Start page: 71;
Date: 2010;
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Keywords: genodermatoses | dyskeratosis

ABSTRACT
Dyskeratosis congenita, a rare inherited condition, is estimated to occur in 1 in 1 million people.The disease is characterised by a classic triad: nail dystrophy, reticulate skin pigmentation, andoral leukoplakia. These patients may also exhibit variable extend of pulmonary, gastrointestinal,genitourinary, cerebral, and dental involvement. Early mortality is often associated with bonemarrow failure, infections, fatal pulmonary complications, or malignancy. More than 200individuals have been reported in the literature with dyskeratosis congenita. Since most cases areinherited as X linked recessive disorder DKC in female is rare. Here we present a case ofdyskeratosis congenita in a 16 year old female patient, who presented all features of classic triadand few additional skeletal features as well.
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