Sudoscan as a noninvasive tool to assess sudomotor dysfunction in patients with Fabry disease: results from a case–control study

Abstract

Hypohidrosis is a frequent and early symptom in patients with Fabry disease. Studies have reported improved sweating in patients treated with enzyme-replacement therapy. A new method, Sudoscan, has been developed that is noninvasive, is quantitative, and can quickly evaluate sweat gland function. It is based on the electrochemical reaction between sweat chlorides and stainless-steel electrodes in contact with the palms and soles. The aim of our study was to evaluate the Sudoscan as a tool to assess sudomotor dysfunction in patients with Fabry disease. Consecutive patients were prospectively recruited who had a diagnosis of Fabry disease, which had been confirmed genetically and/or by measurement of α-galactosidase activity in leukocytes. Healthy controls, matched (1:1) for age and sex, were also enrolled. Test results were expressed immediately as electrochemical skin conductance (ESC, µS) for hands and feet. Sudomotor dysfunction was considered absent, moderate, or severe if the ESC measured on the feet was >60 µS, between 60 and 40 µS, or <40 µS, respectively. Among the 18 patients, 11 had hypohidrosis or anhidrosis. Hand and feet ESCs were significantly lower in patients compared to their controls (P=0.0015 and P=0.0047, respectively). Among patients, 8/18 (44.5%) had a sudomotor dysfunction, moderate in three and severe in five cases. Hand and feet ESCs were significantly lower in those with hypohidrosis/anhidrosis compared to those without (P=0.0014 and P=0.0056, respectively). This study showed that Sudoscan provided a quick, noninvasive, and quantitative measurement of sudomotor function in Fabry disease patients.

Electrochemical skin conductance by Sudoscan®: a new tool to predict intradialytic hypotension

“…The Sudoscan ® procedure is an easy-to-perform evaluation, initially developed for diabetic populations in order to study early-stage peripheral polyneuropathy, small nervous fibre pathology and autonomic neuropathy [ 19 ]. It has also been tested and validated in non-diabetic populations with small nervous fibre pathology, such as patients with familial amyloid and Fabry disease [ 20 ]. A Chinese study of 2833 diabetic patients showed that the Sudoscan ® device could be used on patients with chronic kidney disease (CKD) in order to detect incipient uraemic neuropathy and its long-term evolution towards CKD [ 21 ].…”

Global warming, heat-related illnesses, and the dermatologist

“…Protean clinical signs but hypohidrosis often an early manifestation. Secondary to abnormal autonomic innervation or to eccrine gland dysfunction from lysosomal storage ( Sahuc et al, 2016 ) Hypermobile Ehlers-Danlos syndrome (formerly Type III). Dysautonomia with orthostatic intolerance and other symptoms of dysautonomia.…”

“…Hypohidrosis, neuropathic pain, and angiokeratomas can be early features of Fabry disease ( Lidove et al, 2012 , Sahuc et al, 2016 ), caused by a deficiency of the lysosomal enzyme alpha-galactosidase A. The clinical manifestations of Fabry disease are broad and variable, which can lead to delays in diagnosis and the initiation of corrective enzyme replacement therapy ( Lidove et al, 2012 ).…”

Electrochemical Skin Conductance Alterations during Spinal Cord Stimulation: An Experimental Study

“…Sudorimetry, i.e., measuring the function of sweat gland innervation, specifically evaluates the peripheral sympathetic system [31]. In contrast to quantitative sudomotor axon reflex tests or skin biopsies, the Sudoscan TM is a simple, non-invasive sudomotor test that allows measurement of the sweat gland function [32]. It is easy to perform, has a good reproducibility [33,34], can be rapidly conducted, and is very well tolerated (i.e., not uncomfortable), which are crucial aspects to implement this technique in clinical practice [35].…”