Skin-impedance in Fabry Disease: a prospective, controlled, non-randomized clinical study

Area of Science:

• Biomedical Engineering
• Dermatology
• Neurology

Background:

• Fabry disease is a genetic disorder affecting sweating.
• Enzyme replacement therapy (ERT) has shown potential in improving sweating in Fabry disease.
• Skin impedance is a measure of skin moisture, utilized in assessing skin integrity.

Purpose of the Study:

• To evaluate the utility of the portable dynamic dermal impedance monitor (DDIM) system in detecting skin moisture differences in Fabry disease patients.
• To compare skin impedance measurements between Fabry disease patients and healthy controls.
• To assess the impact of enzyme replacement therapy (ERT) on skin moisture in Fabry disease.

Main Methods:

• Skin impedance measurements were taken using the DDIM system in 22 hemizygous Fabry disease patients (22 on ERT, 5 ERT-naive) and 22 healthy controls.
• Force-compensated skin-moisture values were analyzed using four outcome measures: 100th reading, rate of change, average of 60-110th readings, and overall average.
• Statistical analysis was performed to compare skin moisture between groups.

Main Results:

• Significant differences in skin moisture were observed between Fabry disease patients and healthy controls across all outcome measures (p < 0.0001).
• No significant difference in skin moisture was found between Fabry disease patients receiving ERT and those who were ERT-naive.
• Elevated skin impedance values were noted in a limited number of dermatomes two days post-enzyme infusion.

Conclusions:

• The DDIM system is a portable, user-friendly, and time-efficient clinical tool for assessing skin moisture.
• The DDIM system successfully differentiates skin moisture levels in Fabry disease patients compared to healthy individuals.
• Further investigation into localized skin impedance changes post-infusion may be warranted.