Acrogeria

Orpha code: 2500OMIM code: 201200

Definition

A rare premature aging syndrome characterized by atrophy of the skin and subcutaneous tissue involving predominantly the distal parts of the extremities, resulting in prematurely aged appearance of the hand and feet. Another prominent feature is the characteristic facies with hollow cheeks, beaked nose, and owl-like eyes. Additional, non-dermatological manifestations, like bone anomalies have been described in some patients. Mode of inheritance has not been definitively established.

Disease data
Classification

Malformation syndrome

Synonyms
Acrogeria, Gottron type
Akrogeria, typu Gottrona
Akrometageria
Zespół Gottrona
Acrometageria
Gottron syndrome
ORPHA code
2500
OMIM code
201200
ICD10 code
L90.8
ICD11 code
LD2B

No additional description.

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