Brain Advance Access published online on January 16, 2006
Brain, doi:10.1093/brain/awl010
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1 Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan; Section of Neurology, Department of Internal Medicine, Far Eastern Memorial Hospital, Taipei, Taiwan
* To whom correspondence should be addressed. To understand the clinical significance and mechanisms of cutaneous denervation in systemic lupus erythematosus (SLE), we assessed intraepidermal nerve fibre (IENF) density of the distal leg in 45 SLE patients (4 males and 41 females, aged 38.4 ± 13.6 years) and analysed its correlations with pathology, lupus activity, sensory thresholds and electrophysiological parameters. Compared with age- and gender-matched control subjects, SLE patients had lower IENF densities (3.08 ± 2.17 versus 11.27 ± 3.96 fibres/mm, P < 0.0001); IENF densities were reduced in 38 patients (82.2%). Pathologically, 11 patients (24.4%) were found to have definite cutaneous vasculitis; the severity and extent of cutaneous vasculitis were correlated with IENF densities. Patients with active lupus had even lower IENF densities than those with quiescent lupus (1.86 ± 1.37 versus 4.15 ± 2.20 fibres/mm, P = 0.0002). By linear regression analysis, IENF densities were negatively correlated with the SLE disease activity index (r = 0.527, P = 0.0002) and cumulative episodes of lupus flare-up within 2 years before the skin biopsy (r = 0.616, P = 0.0014). Clinically, skin denervation was present not only in the patients with sensory neuropathy but also in the patients with neuropsychiatric syndrome involving the CNS. SLE patients had significantly elevated warm threshold temperatures (P = 0.003) and reduced cold threshold temperatures (P = 0.048); elevated warm threshold temperatures were associated with the reduced IENF densities (P = 0.032). In conclusion, cutaneous vasculitis and lupus activities underlie skin denervation with associated elevation of thermal thresholds as a major manifestation of sensory nerve injury in SLE.
Received September 30, 2005
Revised November 28, 2005
Accepted December 20, 2005
Article
Skin denervation and cutaneous vasculitis in systemic lupus erythematosus
Ming-Tsung Tseng 1,
Song-Chou Hsieh 2,
Chia-Tung Shun 3,
Kuang-Lun Lee 2,
Chun-Liang Pan 4,
Whei-Min Lin 5,
Yea-Hui Lin 6,
Chia-Li Yu 7,
and
Sung-Tsang Hsieh 8 *
2 Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
3 Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan; Department of Forensic Medicine, National Taiwan University College of Medicine, Taipei, Taiwan
4 Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan; Helen Wills Neuroscience Institute, University of California, Berkeley, CA, USA
5 Department of Anatomy and Cell Biology, National Taiwan University College of Medicine, Taipei, Taiwan
6 Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan
7 Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan; Graduate Institute of Molecular Medicine, National Taiwan University College of Medicine, Taipei, Taiwan
8 Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan; Department of Anatomy and Cell Biology, National Taiwan University College of Medicine, Taipei, Taiwan
Sung-Tsang Hsieh, E-mail: sthsieh{at}ha.mc.ntu.edu.tw
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