Dyskeratosis congenita: Oral hyperkeratosis in association with lichenoid reaction

T.P.B. Handley, G.R. Ogden

    Research output: Contribution to journalArticle

    15 Citations (Scopus)

    Abstract

    Dyskeratosis congenita is an inherited disorder that usually presents in males, consisting of a triad of leukoplakia of the mucous membranes, nail dystrophy and skin pigmentation. Whilst most cases are X-linked, autosomal dominant and recessive forms have also been reported. The significance of the condition lies in premature mortality arising from either bone marrow failure or malignant change within the areas of mucosal leukoplakia. We present a case (X-linked recessive form) where the classic triad of signs were present, along with the development of lichenoid reaction in the buccal and labial mucosa. This is believed to be the first case in which such a reaction has been noted together with the oral hyperkeratosis. The identification of a white patch within the mouth of a child, in the absence of any other obvious cause must arouse suspicion of this rare condition. All clinicians should be aware of this rare genetic disorder so that early referral can be made and appropriate management instigated.
    Original languageEnglish
    Pages (from-to)508-512
    Number of pages5
    JournalJournal of Oral Pathology and Medicine
    Volume35
    Issue number8
    DOIs
    Publication statusPublished - Sep 2006

    Keywords

    • dyskeratosis congenita
    • leukoplakia
    • lichen planus
    • Oral cancer
    • Oral mucosa

    Fingerprint Dive into the research topics of 'Dyskeratosis congenita: Oral hyperkeratosis in association with lichenoid reaction'. Together they form a unique fingerprint.

  • Cite this