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NCT Number: NCT06177353

Study of the Blood and Skin Immunological Profile of Patients With Recessive Dystrophic Epidermolysis Bullosa: in Vivo Analysis and the Impact of Placental Stem Cells in Vitro

Patients with recessive dystrophic epidermolysis bullosa (RDEB) suffer from acute and chronic post-bullous wounds along with impaired skin healing. These issues are attributed not only to mucocutaneous fragility and abnormal healing directly related to quantitative and/or qualitative constitutional abnormalities of collagen VII but also to a contingent cutaneous and systemic inflammatory component. This inflammatory aspect contributes to the perpetuation of skin lesions and delayed healing. Our primary objective is to define the systemic immunological/inflammatory signature of patients with RDEB with an aim to develop a strategy that involves using stem cells with high immunomodulatory/anti-inflammatory capacity such as allogeneic placental stem cells (WJ-MSCs and trophoblasts).

Recruiting

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Key information

Age range

18 year–80 year

Sex eligibility

All sexes

Study type

Observational

Primary location

Hopital Saint Louis - Service de chirurgie plastique reconstructrice et esthétique et traitement chirurgical des brûlés, Paris, France

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Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

EBDR patients :

  • Patients aged 18 to 80 years old
  • Clinically, histologically, and/or genetically confirmed intermediate, reversed or generalized, moderate to severe EBDR

Healthy controls :

  • Adults aged 18 to 80 years old
  • PBMC healthy donors: subjects who have donated blood to the EFS according to the indication criteria who have consented to the use of their samples for research purposes.
  • Healthy skin biopsy donors: subjects undergoing abdominoplasty scheduled in plastic surgery and who have given their consent for the collection of a skin biopsy from post-operative abdominoplasty skin remnants.
  • Healthy donors of bandages soiled with exudates from cutaneous wounds: subject consulting a plastic surgery department as part of their usual post-operative follow-up, who have given their consent for the collection of one of their dressings during their usual during their usual renewal.

For all subjects :

  • Free, informed, written consent, signed by the person and the investigator no later than the day of inclusion and before any examination carried out as part of the study.
  • Person affiliated or benefiting from a social security scheme

Exclusion criteria

EBDR patients :

  • EBH with no definite diagnosis or other than EBDR intermediate, reversed or generalized
  • Systemic anti-inflammatory or immunosuppressive therapy for less than one month
  • Refusal of skin biopsy

Healthy controls :

  • Acute or chronic systemic or cutaneous inflammatory disease at the time of sampling
  • Current immunosuppressive anti-inflammatory treatment in the month prior to sampling

For all subjects:

  • Persons under guardianship or curatorship, or deprived of their liberty by judicial or administrative decision
  • Patients receiving State Medical Aid
  • Pregnant or breast-feeding women

Treatment and study plan

Sampling

Other

Blood sampling Skin biopsy Collection of soiled bandages

Primary outcomes

  1. Analysis of the cellular (PBMC) and cytokine (serum) immunological signature in the peripheral blood of EBDR subjects and healthy controls.

    Time frame: Up to 1 year

Secondary outcomes

  1. Cellular, cytokine, and lipid immunological signatures of immune cells

    Time frame: Up to 1 year

    Immune cells are purified from skin biopsies and soiled dressings of EBDR patients and healthy controls. Individual data will be correlated to the severity grade of EBD.

  2. Immunogenicity and impact of WJ-MSCs and trophoblasts

    Time frame: Up to 1 year

    Immunogenicity and impact of WJ-MSCs and trophoblasts on cellular, cytokine, and lipid immunological signatures, activation, and proliferation of peripheral or cutaneous immune cells from EBDR patients and healthy controls, using in-vitro models.

  3. Immunogenicity and impact of EV/Ex

    Time frame: Up to 1 year

    Immunogenicity and impact of EV/Ex on cellular, cytokine, and lipid immunological signatures, activation, and proliferation of peripheral or cutaneous immune cells of EBDR patients and healthy controls, using in-vitro models.

Study contacts

Contact information is provided by the study sponsor or research team.

Emmannuelle Bourrat, Pr

CONTACT

[email protected]

+33142499090

Jérôme Lambert, Pr

CONTACT

[email protected]

+33142499742

Sponsors and collaborators

Lead sponsor

Assistance Publique - Hôpitaux de Paris

Other

Registry information

Official study title

Etude du Profil Immunologique Sanguin et cutané Des Patients Atteints d'épidermolyse Bulleuse Dystrophique récessive : Analyses in Vivo et Impact Des Cellules Souches Placentaires in Vitro

Acronym: ISTRADEB

Important dates

Study start
2024
Primary completion
2025
Study completion
2025
First posted
Dec 20, 2023
Registry last updated
Jul 17, 2024

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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