Observational Study of a Cohort of Patients With Hereditary Epidermolysis Bullosa

Observational Study of a Cohort of Patients With Hereditary Epidermolysis Bullosa That Come for Their Annual/Biannual Check up at This Medical Rare Disease Reference Centers (MRDRC) of This Disease in France and Belgium

Hereditary Epidermolysis Bullosa (EBH) are rare dermatologic diseases characterized by cutaneous and mucosa fragility. Oral manifestations of few small cohort have been published. The main objective of this multicentric cohort study first in Europe was to report the oral status of these patients that were consulted in the MRDRC of this disease in Nice (France), Toulouse (France) and Louvain (Belgium). Then a correlation between the oral characteristics and the EBH type will be made, in order to facilitate the management of patient care and the prevention program that can be established to improve their oral health.

Study Overview

• Status: Completed
• Conditions: Epidermolysis Bullosa
• Intervention / Treatment: Other: clinical exam

• Study Type: Observational
• Enrollment (Actual): 41

Contacts and Locations

Study Locations

• France
  • Nice, France, 06000
    • Nice hospital
  • Toucy, France, 33000
    • Toulouse University

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 7 months to 78 years (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: N/A
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

All patients with EBH are recruted from the 2 MRDRC of the disease in France and the one of Belgium, the dental visit was/is organized for each visit/follow up of the patient in the center (annual or bianual visit)

Description

Inclusion Criteria:

• patient with EBH
• patient consent for examination and use the clinical data for publication purpose

Exclusion Criteria:

-

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
EBH	Other: clinical exam; clinical oral examination

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Dental structural abnormalities and/or caries	number of defect /dental caries dor each toth	1 day

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
gingival biotype	clinical observation of gingiva (thin or thick)	1 day
gingival status	observation and classification of the gingiva (healthy, inflammed or hyperplastic)	1 day
plaque and gingival index	registration of plaque and gingival index according to the Loë and Silness method	1 day
oral lesion	number and localisation of the blister/scars/flanges scars on a sketch of a mouth	1 day

Collaborators and Investigators

• Sponsor: Centre Hospitalier Universitaire de Nice

Study record dates

Study Major Dates

• Study Start (Actual): January 4, 2017
• Primary Completion (Actual): December 11, 2019
• Study Completion (Actual): December 11, 2019

Study Registration Dates

• First Submitted: December 31, 2019
• First Submitted That Met QC Criteria: January 2, 2020
• First Posted (Actual): January 3, 2020

Study Record Updates

• Last Update Posted (Actual): January 3, 2020
• Last Update Submitted That Met QC Criteria: January 2, 2020
• Last Verified: December 1, 2019

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Skin Diseases; Congenital Abnormalities; Genetic Diseases, Inborn; Skin Diseases, Genetic; Skin Diseases, Vesiculobullous; Skin Abnormalities; Epidermolysis Bullosa
• Other Study ID Numbers: 19-ODONTO-01

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No