LMNA-related cardiocutaneous progeria syndrome

Orpha code: 363618OMIM code:

Definition

LMNA-related cardiocutaneous progeria syndrome is a rare, genetic, premature aging syndrome characterized by adulthood-onset cutaneous manifestations that result in a prematurely aged appearance (i.e. premature thinning and graying of scalp hair, loss of subcutaneous fat, tightening of skin) associated with prominent cardiovascular manifestations, such as accelerated atherosclerosis, calcific valve disease, and cardiomyopathy. Patients present loss of eyebrows and eyelashes in childhood and have a predisposition to develop malignancies.

Disease data
Classification

Disease

Synonyms
LCPS
LCPS
ORPHA code
363618
OMIM code
-
ICD10 code
E34.8
ICD11 code
-

No additional description.

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