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Carvajal syndrome

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Disease definition

A rare genetic ectodermal dysplasia syndrome characterized by woolly hair (presenting at birth), palmoplantar keratoderma (developing in the first year of life) and dilated cardiomyopathy with predominant left ventricle involvement (developing in childhood) which can lead to life-threatening heart failure in childhood or adolescence.

ORPHA:65282

Classification level: Disorder

Synonym(s):
  • Wooly hair-palmoplantar hyperkeratosis-dilated cardiomyopathy syndrome
  • Wooly hair-palmoplantar keratoderma-dilated cardiomyopathy syndrome
  • Woolly hair-palmoplantar keratoderma-dilated cardiomyopathy syndrome
  • KWWH type II
  • Keratoderma with woolly hair type II
  • Woolly hair-palmoplantar hyperkeratosis-dilated cardiomyopathy syndrome

Prevalence: <1 / 1 000 000

Inheritance: Autosomal dominant, Autosomal recessive

Age of onset: Childhood, Infancy, Neonatal

ICD-10: I42.0

ICD-11: BC43.6

OMIM: 605676 615821

UMLS: C1854063

MeSH: C535581

GARD: 5595

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands, Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2019.pdf) - Orphanet Urgences
Disease review articles
Clinical genetics review
English (2024) - GeneReviews
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