Dyskeratosis congenita in two male cousins

Dyskeratosis congenita is reported in two male Singaporean Chinese cousins. The family pedigree is presented. Lichen planus or lichenoid lesions, instead of leukoplakia, was the manifestation in the oral cavity. The mode of inheritance and the differential diagnosis of Fanconi's anaemia are discussed. Dental findings are emphasised as such information is lacking in previously reported cases. One of the subjects expired of aplastic anaemia, which together with oral malignancy, is one fatal complication of this disease.