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Start free with EleplanPosterior polymorphous corneal dystrophy
ORPHA:98973Disease
Also called PPCD · Posterior polymorphous dystrophy · Schlichting dystrophy
What it is
A rare mild subtype of posterior corneal dystrophy characterized by small aggregates of apparent vesicles bordered by a gray haze at the level of Descemet membrane, generally with no effect on vision.
Key facts
- Prevalence
- 1-9 / 100 000 (Czech Republic)
- Age of onset
- Childhood
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Sometimes5–29%
8Rare1–4%
10- Blurred vision
- Chorioretinal degeneration
- Corneal opacity
- Ectopia pupillae
- Esotropia
- Glaucoma
- Lacrimation abnormality
- Ocular hypertension
and 2 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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