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

BMC Neurol. 2008 Nov 6:8:41. doi: 10.1186/1471-2377-8-41.

Abstract

Background: We previously demonstrated improved sweating after enzyme replacement therapy (ERT) in Fabry disease using the thermo-regularity sweat and quantitative sudomotor axon reflex tests. Skin-impedance, a measure skin-moisture (sweating), has been used in the clinical evaluation of burns and pressure ulcers using the portable dynamic dermal impedance monitor (DDIM) system.

Methods: We compared skin impedance measurements in hemizygous patients with Fabry disease (22 post 3-years of bi-weekly ERT and 5 ERT naive) and 22 healthy controls. Force compensated skin-moisture values were used for statistical analysis. Outcome measures included 1) moisture reading of the 100th repetitive reading, 2) rate of change, 3) average of 60-110th reading and 4) overall average of all readings.

Results: All outcome measures showed a significant difference in skin-moisture between Fabry patients and control subjects (p < 0.0001). There was no difference between Fabry patients on ERT and patients naïve to ERT. Increased skin-impedance values for the four skin-impedance outcome measures were found in a small number of dermatome test-sites two days post-enzyme infusions.

Conclusion: The instrument portability, ease of its use, a relatively short time required for the assessment, and the fact that DDIM system was able to detect the difference in skin-moisture renders the instrument a useful clinical tool.

Publication types

  • Controlled Clinical Trial
  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Analysis of Variance
  • Case-Control Studies
  • Electric Impedance
  • Fabry Disease / pathology*
  • Fabry Disease / physiopathology*
  • Humans
  • Longitudinal Studies
  • Middle Aged
  • Skin / drug effects
  • Skin / physiopathology*
  • Sweating / drug effects
  • Sweating / physiology
  • alpha-Galactosidase / biosynthesis
  • alpha-Galactosidase / therapeutic use*

Substances

  • alpha-Galactosidase