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Start free with EleplanPeriodontal Ehlers-Danlos syndrome
ORPHA:75392Disease
Also called EDS VIII · Ehlers-Danlos syndrome type 8 · Ehlers-Danlos syndrome, periodontitis type · Periodontal EDS · pEDS
What it is
A rare type of Ehlers-Danlos syndrome characterized by childhood or adolescence onset of severe, intractable periodontitis, lack of attached gingiva, and presence of pretibial plaques. Additional manifestations are easy bruising, hypermobility mainly of the distal joints, skin hyperextensibility and fragility, abnormal scarring, recurrent infections, hernias, marfanoid facial features, acrogeria, and prominent vasculature.
Key facts
- Age of onset
- Childhood
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- 1-9 / 1 000 000 (at birth, Europe)Ehlers-Danlos 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
Very common80–99%
4- Bruising susceptibilityDiagnostic criterion
- Erythematous plaqueDiagnostic criterion
- Pretibial hyperpigmentationDiagnostic criterion
- Severe periodontitisDiagnostic criterion
Common30–79%
18- Agenesis of permanent teeth
- Anxiety
- Atrophic scarsDiagnostic criterion
- Atrophy of alveolar ridges
- Enamel hypoplasia
- Fragile skinDiagnostic criterion
- Gingival recession
- Headache
- Hoarse voice
- Hyperextensible skinDiagnostic criterion
- Hypoplasia of the dental root
- Joint hypermobility
- Microdontia
- Poor wound healing
- Pulp calcification
- Recurrent infectionsDiagnostic criterion
- Thin skin
- Varicose veins
Sometimes5–29%
9- Abnormally high-pitched voice
- Depression
- Distal joint hypermobilityDiagnostic criterion
- HerniaDiagnostic criterion
- Joint dislocation
- Leukodystrophy
- Premature loss of primary teeth
- Prominent superficial blood vesselsDiagnostic criterion
and 1 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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