We have located links that may give you full text access.
ENGLISH ABSTRACT
JOURNAL ARTICLE
REVIEW
[Alterations in nails and teeth as a clue for genodermatoses].
BACKGROUND: There are about 10,000 monogenic diseases and around 30% demonstrate alterations in the skin and its appendages. As there are so many genetic different skin diseases, clear diagnosis is often very difficult.
AIM: The goal of this review is to give the clinicians some key features on nails and teeth which might help to identify rare genodermatoses.
DISCUSSION: In the daily work genodermatoses manifest more commonly as incomplete or oligosymptomatic syndromes than as complete symptom complexes. To diagnose a rare disorder in such situations, a knowledge of key features which are characteristic for a genodermatoses is essential, so that a diagnosis can be advanced and the underlying gene defect identified. Changes in nails and teeth sometimes may be useful as diagnostic key features. Both structures originate from ectoderm and therefore they often appear in combination in diseases with major ectodermal malformations. Enamel defects resembling the lines of Blaschko are highly suggestive for focal dermal hypoplasia, even if other important signs and symptoms are missing. Enamel defects combined with gingival fibromas are highly suggestive for tuberous sclerosis. On the other side, triangular lunulae with malformation and dystrophy of the nail plate suggests nail-patella syndrome.
AIM: The goal of this review is to give the clinicians some key features on nails and teeth which might help to identify rare genodermatoses.
DISCUSSION: In the daily work genodermatoses manifest more commonly as incomplete or oligosymptomatic syndromes than as complete symptom complexes. To diagnose a rare disorder in such situations, a knowledge of key features which are characteristic for a genodermatoses is essential, so that a diagnosis can be advanced and the underlying gene defect identified. Changes in nails and teeth sometimes may be useful as diagnostic key features. Both structures originate from ectoderm and therefore they often appear in combination in diseases with major ectodermal malformations. Enamel defects resembling the lines of Blaschko are highly suggestive for focal dermal hypoplasia, even if other important signs and symptoms are missing. Enamel defects combined with gingival fibromas are highly suggestive for tuberous sclerosis. On the other side, triangular lunulae with malformation and dystrophy of the nail plate suggests nail-patella syndrome.
Full text links
Related Resources
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.
By using this service, you agree to our terms of use and privacy policy.
Your Privacy Choices
You can now claim free CME credits for this literature searchClaim now
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app