Hypertrichosis

 

Congenital hypertrichosis lanuginosa presents as a confluent, generalized overgrowth of silvery blonde to gray lanugo hair at birth or in early infancy. It is rare (1 in 1 billion) and thought to occur as an autosomal dominant trait with variable expressivity. In most cases, other than possibly anomalous dental eruptions, children are otherwise healthy. The hair may persist, increase, or decrease with age.

There are several other congenital disorders associated with generalized hypertrichosis, but none that are so evenly distributed as congenital hypertrichosis lanuginosa  Patients with the autosomal dominant Ambras syndrome or hypertrichosis universalis congenita present with much longer, thicker hair, with accentuation over the entire face, ears, and shoulders. Associated facial dysmorphism and dental anomalies are common. Members of the five-generation family described with congenital generalized hypertrichosis also have excess terminal hair on the face and upper body, more severe in men than women in keeping with the X-linked dominant inheritance. Patients with the autosomal dominant (rarely autosomal recessive) gingival fibromatosis frequently have hypertrichosis, mostly on the face, eyebrows, limbs, and upper back, along with seizures and oligophrenia. Hypertrichosis may be delayed until puberty, although gingival fibromatosis in this situation usually appears with the emergence of the primary, versus secondary, teeth.