Hereditary sclerosing poikiloderma, Weary type

Orpha code: 221039OMIM code: 173700

Definition

A rare genetic skin disease characterized by generalized poikiloderma with marked accentuation in flexural regions and on extensor surfaces, sclerosis of palms and soles, and linear and reticulated hyperkeratotic and sclerotic bands in the axilla and the antecubital and popliteal fossae. Subcutaneous calcification, finger clubbing, Raynaud phenomenon, and cardiac abnormalities (such as severe aortic stenosis) have also been reported.

Disease data
Classification

Disease

ORPHA code
221039
OMIM code
173700
ICD10 code
Q82.8
ICD11 code
EC10

No additional description.

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