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