Dyskeratosis Congenita Preceded By Severe Aplastic Anemia: Report of One Case

Dyskeratosis congenita is a rare hereditary disease which usually manifests with skin hyperpigmentation, nail dystrophy, and leukoplakia of the mucous membrane (triad). This report describs a six-year-old boy with severe aplastic anemia who was later diagnosed to have dyskeratosis congenita. His unusual presentation was pancytopenia followed by leukoplakia of the tongue, hyperpigmentation of the skin and dystrophy of the nails. Treatment with horse anti-human lymphocyte immunoglobulin (ALG) for his aplastic anemia was not effective.