Hereditary gingival fibromatosis with hemophilia B.

This work presents a case report of a generalized form of hereditary gin­ gival fibromatosis with hemophilia B as an accompanying disease. In the family of proband, consisting of 28 members, fibromatosis was present in 9 (4 males and 5 females). The pedigree analysis confirmed that gingival fibro­ matosis was transmited through three generations as an autosomal dominant trait. Neither proband, nor any other family member, showed other abnorma­ lities. Blood coagulation tests reveald hemophilia B (Christmas disease) in the proband. The coagulogram showed prolonged kaolin cephalin time (50 se­ conds) and low concentration of factor IX (F IX 18%). The case report sug­ gests that hemophilia B should be included in the list of diseases associated with gingival fibromatosis.