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Anhidrotic and achromians lesions in incontinentia pigmenti

Identifieur interne : 000737 ( Istex/Corpus ); précédent : 000736; suivant : 000738

Anhidrotic and achromians lesions in incontinentia pigmenti

Auteurs : Celia Moss ; P. Ince

Source :

RBID : ISTEX:0EF0089975CF8F3B6944E422396AA9E0FC068709

English descriptors

Abstract

Three women subsequently shown to have incontinentia pigmenti (IP) presented with white hairless streaks on the limbs as the predominant cutaneous abnormality. Seven other patients with IP diagnosed in infancy were reviewed to establish the frequency of this sign. It was found in all of them, and in the otherwise unaffected mother of three affected girls. A focal absence of sweating in these lesions is reported here for the first time. There are other similarities with anhidrotic ectodermal dysplasia, suggesting a genetic overlap between these two X‐linked conditions.

Url:
DOI: 10.1111/j.1365-2133.1987.tb04903.x

Links to Exploration step

ISTEX:0EF0089975CF8F3B6944E422396AA9E0FC068709

Le document en format XML

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<p>Three women subsequently shown to have incontinentia pigmenti (IP) presented with white hairless streaks on the limbs as the predominant cutaneous abnormality. Seven other patients with IP diagnosed in infancy were reviewed to establish the frequency of this sign. It was found in all of them, and in the otherwise unaffected mother of three affected girls. A focal absence of sweating in these lesions is reported here for the first time. There are other similarities with anhidrotic ectodermal dysplasia, suggesting a genetic overlap between these two X‐linked conditions.</p>
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<abstract lang="en">Three women subsequently shown to have incontinentia pigmenti (IP) presented with white hairless streaks on the limbs as the predominant cutaneous abnormality. Seven other patients with IP diagnosed in infancy were reviewed to establish the frequency of this sign. It was found in all of them, and in the otherwise unaffected mother of three affected girls. A focal absence of sweating in these lesions is reported here for the first time. There are other similarities with anhidrotic ectodermal dysplasia, suggesting a genetic overlap between these two X‐linked conditions.</abstract>
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