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Start free with EleplanCleft lip/palate-ectodermal dysplasia syndrome
ORPHA:3253Malformation syndrome
Also called CLPED1 · Cleft lip/palate-syndactyly-pili torti syndrome · Syndactyly-ectodermal dysplasia-cleft/lip palate · Zlotogora-Ogur syndrome
What it is
Zlotogora-Ogur syndrome is an ectodermal dysplasia syndrome characterized by hair, skin and teeth anomalies, facial dysmophism with cleft lip and palate, cutaneous syndactyly and, in some cases, intellectual disability.
Key facts
- Age of onset
- Infancy, Neonatal
- Inheritance
- Autosomal recessive
- Classified as
- Malformation syndrome
Recorded for the broader condition
- Prevalence
- 6-9 / 10 000Ectodermal dysplasia syndrome
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Signs and symptoms
Common30–79%
26- Abnormal dental morphology
- Abnormality of speech or vocalization
- Abnormality of the dentition
- Alopecia
- Aplasia/Hypoplasia of the eyebrow
- Bilateral single transverse palmar creases
- Carious teeth
- Downslanted palpebral fissures
- Dystrophic fingernails
- Dystrophic toenail
- Highly arched eyebrow
- Hypogonadism
- Hypoplasia of the zygomatic bone
- Intellectual disability
- Micrognathia
- Midface retrusion
- Nail dystrophy
- Pili torti
- Protruding ear
- Recurrent respiratory infections
- Small scrotum
- Sparse hair
- Sparse lateral eyebrow
- Synophrys
- Wide intermamillary distance
- Wide nasal bridge
Sometimes5–29%
10- Abnormal dermatoglyphics
- Abnormality of dental enamel
- Abnormality of the ureter
- Anodontia
- EEG abnormality
- Hyperlordosis
- Hypodontia
- Hypohidrosis
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.
Gene
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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