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Метеликоподібна пігментна дистрофія

ORPHA:99001· ICD-10 H35.5· Butterfly-shaped pigment dystrophy

Визначення(English summary)

A rare patterned dystrophy of the retinal pigment epithelium characterized by abnormal accumulation of lipofuscin in a butterfly-shaped distribution at the retinal pigment epithelium level. Patients manifest with a slowly progressive loss of vision that often only becomes apparent in old age.

Поширеність
<1 / 1 000 000
Успадкування
Autosomal dominant
Вік початку
Adult