Netherton Syndrome, a general introduction.

Netherton Syndrome is also known as the Comel Netherton Syndrome. In 1958, Earl W Netherton describes a first case of a four year old girl. This girl has a very red and scaly skin, and she also has an unusual hair disorder. He calls this strange hair growth ‘Bamboo Hair’, as that is what he saw through his microscope: a bamboo structure.

Nine years earlier, the Italian dermatologist Comel described a 23 year old woman with round and scaly patches on her skin, which he called ‘Ichthyosis Linearis Circumflexa’. The symptoms of these two patients were found to be related. Similar cases were described in the ‘60’s.

Netherton Syndrome is a very rare disease. In Holland there is a population of one in a million people, who is affected with the syndrome.  From online information, we know the same number of affected people in Sweden is also one in a million.  Dermatologists suspect that there are more patients with this skindisorder, they think that the diagnose is often missed. Therefore it is important to be informed about this disease. Netherton Syndrome can cause an early infant death, as their skin is very vulnerable.

Netherton Syndrome, and also other forms of Ichthyosis, require very good skin care. The skin is very dry and there is danger of dehydration. Infections should be closely monitored, because of the weak immunesystem.  You should inform other people about your skin condition and about allergies. Patients, parents, people at school and specialists should all work together.