Hypertrichosis universalis congenita: a separate entity, or the same disease as gingival fibromatosis?

Hypertrichosis universalis congenita is an extremely rare disorder characterized by generalized hypertrichosis. It is generally accepted as being inherited as an autosomal dominant trait with varying expression. Many aspects of this disease are still unknown. Several reports associating hypertrichosis and gingival fibromatosis raise the question of whether they are separate entities or the same disease with different expressions of the underlying process. Hypertrichosis universalis congenita occurred in a 6-year-old girl without known family history. Her facial features were simian-like and her gingiva was moderately hyperplastic. We pose the question of whether or not these phenomena are related.