Periodontal EDS (pEDS)

Please note: The following text cannot and should not replace advice from the patient's healthcare professional(s). Any person who experiences symptoms or feels that something may be wrong should seek individual, professional help for evaluation and/or treatment. This information is for guidance only and is not intended to provide individual medical advice.

What is pEDS?

pEDS is an incredibly rare type of EDS which causes periodontitis which is severe and begins early, individuals have lack of attached gingiva, first generational family who fit the criteria and pretibial plaques.

What is the cause of pEDS?

pEDS is caused by variations in a person’s genes. pEDS is caused by variations in the C1R and C1S genes.

How is pEDS diagnosed?

When diagnosing pEDS a list of common and relevant signs and symptoms of the condition are used. This is referred to as diagnostic criteria and in the case of pEDS it is split into a set of major and minor criteria.

The Major criteria

1. Severe and intractable periodontitis of early onset (childhood or adolescence)
2. Lack of attached gingiva
3. Pretibial plaques
4. Family history of a first-degree relative who meets clinical criteria

The Minor criteria

1. Easy bruising
2. Joint hypermobility, mostly distal joints
3. Skin hyperextensibility43 and fragility, abnormal scarring (wide or atrophic)
4. Increased rate of infections
5. Hernias
6. Marfanoid facial features
7. Acrogeria
8. Prominent vasculature

To meet the diagnostic criteria for pEDS a person must have:

Major criterion 1 (Severe and intractable periodontitis of early onset) or major criterion 2 (Lack of attached gingiva)

Plus

At least two other major criteria and one minor criterion

If a person meets the criteria a genetic test is done to confirm the diagnosis. You can read more about genetic testing for the rare types of EDS here.

How is pEDS managed?

pEDS is managed through addressing the symptoms a person is experiencing. This is best done through being in the care of the NHS National EDS service; a specialised service for adults and children who have or are suspected to have a rare type of EDS.

For information about the service and for referral eligibility please click on the following links:

EDS National Diagnostic Service (based in London) covering the South of England

EDS National Diagnostic Service (based in Sheffield) covering the North of England