Abstract
Three women presented with pale hairless streaks on the limbs as the only remaining sign of incontinentia pigmenti (IP). Seven other women in whom IP had been diagnosed in infancy were re-examined and this sign was present in all and in the otherwise unaffected mothers of three affected girls. Sweating was studied in these lesions because we were struck by the clinical overlap between IP and anhidrotic ectodermal dysplasia (AED) and because the lesions resembled those of hypomelanosis of Ito (HI), which are anhidrotic. Sweating was stimulated locally by iontophoresis of pilocarpine and stained with ferric chloride and tannic acid. Sweating was absent in these lesions. Biopsies showed absent sweat glands and follicles but no abnormal pigmentary incontinence. These are not the changes of post-inflammatory scarring and they differ in distribution, degree and depth from the early inflammatory lesions of IP. It is concluded that maldevelopment of hair follicles and sweat glands is a feature of IP which should be considered as another X-linked anhidrotic ectodermal dysplasia. This view is supported by the recent genetic localization of both conditions to the juxta centromeric region of the X chromosome.
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