A One in a Billion Condition

Congenital hypertrichosis is the condition only 50 people in the world currently live with. One of the most common names for this condition is Ambras Syndrome, though there are very real differences between the two conditions. The conditions manifest as hair growing all over the face and back of the body. This hair is not normal hair, but resembles more closely the fur on an animal’s body. The conditions are two of the rarest in the world and poses serious society risks in children.

When a child is born with congenital hypertrichosis, the hair of the body grows uncontrollably. With extreme amounts of hair on the face and back, the condition commonly leaves the sufferers being called “wolf” children early in life. Aside from the extra hair, the people suffering from this condition have no real medical problems to deal with aside from nasal blockage.

Children and adults with congenital hypertrichosis often grow large amounts of hair in their nasal passages. This hair needs to be removed surgically in order for the person to breathe correctly. The hair will, however, grow back and the surgery may have to be repeated more than one time in a lifetime. Doctors have also tried, in some cases, to remove the excess hair from the body of the patient with laser treatments. The skin often appears green and swollen.

There is no known cure for congenital hypertrichosis. Patients are forced to live with the condition for the rest of their lives. Congenital hypertrichosis is genetic in nature with at least one family living with children who have contracted the condition from their parents.

 The first cases were noted in the 1600s with 34 cases being documented officially. The first case involved a father, two daughters, a son and a grandchild. There are other medical names for congenital hypertrichosis including congenital hypertrichosis lanuginosa, congenital hypertrichosis universalis, hypertrichosis universalis and hypertrichosis lanuginosa universalis.

Hypertrichosis is not to be confused with Hirsutism. Hirsutism occurs in women only. The condition presents similarly to Hypertrichosis, but is related to a congenital defect of the hormones in most cases. Others causes of Hirsutism have been linked to polycystic ovary syndrome, idiopathic hirsutism and congenital adrenal hyperplasia.

While Hypertrichosis is terminal, meaning the patient will suffer from the condition for the rest of their lives, the condition is not known to cause early death. In babies, the condition may take up to two years to appear as physical symptoms. In Ambras syndrome, the hair continues to grow for the rest of life. In Hypertrichosis the hair growth may wane with some hair falling out in adulthood.

The only physical symptom associated with Hypertrichosis is excessive hair growth. This hair growth does not occur on the palms of the hands and feet, mucous membranes and genital areas of the body. There may be physical changes in the teeth and shape of the face and nose. Even more rare cases have presented with extra nipples on the body. The case in question noted six extra nipples on the body.