Wilson's Disease Treated With D-Penicillamine: Characterization of Skin Damage Secondary to Treatment by Measuring Skin Elasticity
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Preparation of forearm molds with SILFLO® silicone.
- Who it may be relevant to
- Registry conditions: Wilson Disease, D-Penicillamine, Effect of D-penicilline on Cutaneous Elastity of Wilson's Patient. Basic parameters: from 12 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- France
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
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Overview
Wilson's disease is a genetic disorder, resulting from an anomaly present on the ATP7B gene located on chromosome 13, causing a progressive accumulation of copper in various organs such as the liver, nervous system and cornea, leading to various hepatic and neurological disorders and a systemic evolution. Currently, the first-line treatment for this disease is D-Penicillamine, which acts by chelation and promotes copper excretion through the urine. Unfortunately, this treatment also has significant side-effects, particularly on the skin. However, the pathogenesis of elastopathy in patients with Wilson's disease has yet to be fully characterized, and needs to be better understood in order to adapt the therapeutic strategy. A silicon mold will be made on Wilson's disease patients, enabling the skin micro-relief to be shaped, and analyzed by confocal laser in comparison with the skin of healthy volunteers.
Interventions
- Other Preparation of forearm molds with SILFLO® silicone
Preparation of forearm molds with SILFLO® silicone (MONADERM, Monaco), CE-marked and already used routinely in dermocosmetics. These molds will, then, be analyzed.
Primary outcome measures
- Skin tension measurement [Time frame: At inclusion]
Secondary outcome measures (2)
- Skin tension indices measurement on silicone casts [Time frame: Year 1]
- Skin tension indice measurement with confocal laser [Time frame: Year 1]
Eligibility criteria
Inclusion criteria
Diseased patients :
- Patient over 12 years old
- Patient with Wilson's disease confirmed by genetic analysis
- Patient followed up in the Wilson's Disease Reference Center for his care
- Patient treated with D-Penicillamine
- Patient with no other known elastic tissue pathology
Healthy volunteers :
- Patient over 12 years old
- Patient followed up in the dermatology department of St Etienne University Hospital
- Patient matched on sex and age with a patient from the "Wilson's disease" group
- Patient with no elastic tissue pathology
All patients :
- Patient affiliated to a national social security
- Patient with written informed consent
Exclusion criteria
All patients:
- Patient not taking a treatment (at investigator's discretion) that may modify skin elasticity
- Patient with pathological lesion(s) on forearm or cheek
- Patient with a potentially active/rejuvenative forearm or cheek treatment
- Patient having applied cream and/or make-up to the areas to be molded (forearm and cheek)
- Patient under guardianship
- Patient unable to follow study procedures
- Pregnant or breast-feeding women
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: Yes
Study design
- Allocation
- Non-randomized
- Model
- Parallel assignment
- Masking
- Open label
- Primary purpose
- Supportive care
Study locations
France · 2 centers
- Hospices Civils de Lyon — Bron
- Hôpital Nord CHU de Saint-Étienne — Saint-Etienne
Identifiers
NCT: NCT06945081 · 25CH057 · ANSM