Letters to the editor
Pili torti et canaliculi and agenesis of the teeth: report of a new "pure" hair-tooth ectodermal dysplasia in a Norwegian family
| The first 150 words of the full text of this article appear below. |
EDITOR
The ectodermal dysplasias comprise a heterogeneous group of
inherited developmental disorders affecting tissue and organs of
ectodermal origin. Their classification was developed by Freire-Maia
and Pinheiro,1 with malformations of the hair, teeth,
nails, and sweat glands as the major criteria. Ectodermal dysplasias
are thus divided into 11 subgroups based on a minimum of two ectodermal
signs with or without other developmental defects.
A Norwegian family with structural hair abnormalities associated with agenesis of the teeth is reported. The mode of inheritance is consistent with an autosomal dominant pattern.
The investigation for hair and tooth abnormalities in the family
started with a then 16 year old boy (IV.3, fig 1), who was under
dermatological treatment for an X linked ichthyosis. Blood lipoprotein
electrophoresis showed increased migration of the low density
lipoprotein (LDL) fraction compared to normal, consistent with steroid
sulphatase deficiency. Interestingly, the blood LDL fraction in his
mother, who
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