Orphanet Rare Disease Ontology

Last uploaded: December 13, 2023
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Preferred Name

Acrokeratosis verruciformis of Hopf
Synonyms

AKV of Hopf
Definitions

A rare, genetic, acrokeratoderma disease characterized by multiple, symmetrical, asymptomatic, skin-colored (rarely, brownish), flat-topped, wart-like papules located on the dorsal aspects of the hands and feet (occasionally found on other parts of the body, such as knees, elbows and forearms), typically associated with palmoplantar punctate keratosis and variable nail involvement (including leukonychia, thickening, ridging, longitudinal striations and splitting). Histology reveals undulating hyperkeratosis, papillomatosis, hypergranulosis, and acanthosis, creating a characteristic 'church spire' appearance, with no acantholysis nor dyskeratosis associated.
ID

http://www.orpha.net/ORDO/Orphanet_79151
alternative_term

AKV of Hopf
definition

A rare, genetic, acrokeratoderma disease characterized by multiple, symmetrical, asymptomatic, skin-colored (rarely, brownish), flat-topped, wart-like papules located on the dorsal aspects of the hands and feet (occasionally found on other parts of the body, such as knees, elbows and forearms), typically associated with palmoplantar punctate keratosis and variable nail involvement (including leukonychia, thickening, ridging, longitudinal striations and splitting). Histology reveals undulating hyperkeratosis, papillomatosis, hypergranulosis, and acanthosis, creating a characteristic 'church spire' appearance, with no acantholysis nor dyskeratosis associated.
definition_citation

Orphanet
expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=79151
has_age_of_onset

Adult

Infancy

Neonatal
has_inheritance

Autosomal dominant
hasDbXref

UMLS:C0265971

ICD-10:Q82.8

OMIM:101900

ICD-11:EC20.Y

MedDRA:10069445
label

Acrokeratosis verruciformis of Hopf
notation

ORPHA:79151
part_of

http://www.orpha.net/ORDO/Orphanet_183441

http://www.orpha.net/ORDO/Orphanet_79356
prefixIRI

ORDO:Orphanet_79151
prefLabel

Acrokeratosis verruciformis of Hopf
present_in

Worldwide AND has_point_prevalence_range : Unknown
treeView

http://www.orpha.net/ORDO/Orphanet_183441

http://www.orpha.net/ORDO/Orphanet_79356
subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493
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