X LINKED DYSKERATOSIS CONGENITA
\ˈɛks lˈɪŋkt dˌɪskəɹɐtˈə͡ʊsɪs kɒnd͡ʒɪnˈiːtə], \ˈɛks lˈɪŋkt dˌɪskəɹɐtˈəʊsɪs kɒndʒɪnˈiːtə], \ˈɛ_k_s l_ˈɪ_ŋ_k_t d_ˌɪ_s_k_ə_ɹ_ɐ_t_ˈəʊ_s_ɪ_s k_ɒ_n_dʒ_ɪ_n_ˈiː_t_ə]\
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A predominantly X-linked recessive syndrome characterized by a triad of reticular skin pigmentation, nail dystrophy and leukoplakia of mucous membranes. Oral and dental abnormalities may also be present. Complications are a predisposition to malignancy and bone marrow involvement with pancytopenia. (from Int J Paediatr Dent 2000 Dec;10 (4):328-34) The X-linked form is also known as Zinsser-Cole-Engman syndrome and involves the gene which encodes a highly conserved protein called dyskerin.
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