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Acrokeratoelastoidosis of Costa

Orpha number ORPHA38
Synonym(s) AKE
PPKP3
Punctate palmoplantar hyperkeratosis type 3
Punctate palmoplantar keratoderma type 3
Prevalence Unknown
Inheritance Autosomal dominant
Not applicable
Age of onset Adolescent
ICD-10
  • Q82.8
ICD-O -
OMIM
UMLS
  • C0545044
MeSH
  • C535653
MedDRA -
SNOMED CT
  • 111029001

Summary

Acrokeratoelastoidosis of Costa is a rare dermatosis characterized by small, firm papules or plaques (resembling warts) on the sides of the hands and feet. These stationary and asymptomatic lesions appear generally at puberty, or sometimes later. The prevalence is unknown. Both autosomal dominant and sporadic forms have been observed. The histology combines hyperkeratosis and acanthosis. Treatment is not indicated in most patients.


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