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Small-Fibre Neuropathies

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Pruritus

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Abstract

Small-fibre neuropathies (SFN) can be defined as diseases of small nerve fibres: thinly myelinated A-δ and unmyelinated C fibres. The clinical manifestations include autonomic symptoms such as dry eyes, dry mouth, dizziness, constipation, bladder incontinence, sexual dysfunction, excessive sweating, and red or white skin discoloration. Patients also have sensory symptoms that are often their main symptom, including pain, pruritus (in about 2/3 of patients), burning, tingling, or numbness. Patient history and physical examination are considered the most important for diagnosing SFN. Diagnosis can be confirmed with quantitative sensory testing (QST) or with skin biopsies, by reduced intraepidermal nerve fibre (IENF) density measurements in the distal leg. SFN are usually idiopathic but may be associated with various diseases, such as metabolic diseases (diabetes), infectious diseases (HIV, hepatitis C infection), inflammatory diseases (Sjögren syndrome), autoimmune diseases, paraneoplastic syndromes, or genetic diseases (Fabry disease). SFN lead to a significant reduction in the overall quality of life. Treatment is based on etiology, and symptomatic treatments (e.g., gabapentin, pregabalin, tricyclic antidepressants) can be used as well.

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Abbreviations

AIDS:

Acquired immune deficiency syndrome

BMS:

Primary burning mouth syndrome

DN4:

Douleur Neuropathique 4

HIV:

Human immunodeficiency virus

IENF:

Intraepidermal nerve fibre

NPSI:

Neuropathic pain symptom inventory

PFA:

Paraformaldehyde

PGP9.5:

Protein gene product 9.5

QST:

Quantitative sensory testing

SFN:

Small-fibre neuropathies

TST:

Thermoregulatory sweat testing

References

  1. Peters MJH, Bakkers M, Merkies ISJ, Hoeijmakers JGJ, van Raak EPM, Faber CG. Incidence and prevalence of small-fiber neuropathy: a survey in the Netherlands. Neurology. 2013;81(15):1356–60.

    Article  PubMed  Google Scholar 

  2. Tavee J, Zhou L. Small fiber neuropathy: a burning problem. Cleve Clin J Med. 2009;76(5):297–305.

    Article  PubMed  Google Scholar 

  3. Lacomis D. Small-fiber neuropathy. Muscle Nerve. 2002;26(2):173–88.

    Article  PubMed  Google Scholar 

  4. Misery L, Bodere C, Genestet S, Zagnoli F, Marcorelles P. Small-fibre neuropathies and skin: news and perspectives for dermatologists. Eur J Dermatol EJD. 2014;24(2):147–53.

    PubMed  Google Scholar 

  5. Bernhard JD. Neurogenic pruritus and strange skin sensations. In: Itch, mechanisms and management of pruritus. New York: Mc Graw-Hill; 1994. p. 185–201.

    Google Scholar 

  6. Hoitsma E, Reulen JPH, de Baets M, Drent M, Spaans F, Faber CG. Small fiber neuropathy: a common and important clinical disorder. J Neurol Sci. 2004;227(1):119–30.

    Article  CAS  PubMed  Google Scholar 

  7. Brenaut E, Marcorelles P, Genestet S, Ménard D, Misery L. Pruritus: an underrecognized symptom of small-fiber neuropathies. J Am Acad Dermatol. 2015;72(2):328–32.

    Article  PubMed  Google Scholar 

  8. Lauria G, Bakkers M, Schmitz C, Lombardi R, Penza P, Devigili G, et al. Intraepidermal nerve fiber density at the distal leg: a worldwide normative reference study. J Peripher Nerv Syst JPNS. 2010;15(3):202–7.

    Article  PubMed  Google Scholar 

  9. Stumpf A, Ständer S. Neuropathic itch: diagnosis and management. Dermatol Ther. 2013;26(2):104–9.

    Article  PubMed  Google Scholar 

  10. Marziniak M, Pogatzki-Zahn M, Evers S. Other neurological causes of itch. In: Misery L, Stander S, editors. Pruritus. London Dordrecht Heidelberg New York: Springer; 2010. p. 163–6.

    Google Scholar 

  11. Misery L, Brenaut E, Le Garrec R, Abasq C, Genestet S, Marcorelles P, et al. Neuropathic pruritus. Nat Rev Neurol. 2014;10(7):408–16.

    Article  PubMed  Google Scholar 

  12. Oaklander AL. Common neuropathic itch syndromes. Acta Derm Venereol. 2012;92(2):118–25.

    Article  PubMed  Google Scholar 

  13. Kwatra SG, Stander S, Bernhard JD, Weisshaar E, Yosipovitch G. Brachioradial pruritus: a trigger for generalization of itch. J Am Acad Dermatol. 2013;68(5):870–3.

    Article  PubMed  Google Scholar 

  14. Hovaguimian A, Gibbons CH. Diagnosis and treatment of pain in small-fiber neuropathy. Curr Pain Headache Rep. 2011;15(3):193–200.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Singleton JR, Bixby B, Russell JW, Feldman EL, Peltier A, Goldstein J, et al. The Utah early neuropathy scale: a sensitive clinical scale for early sensory predominant neuropathy. J Peripher Nerv Syst JPNS. 2008;13(3):218–27.

    Article  PubMed  Google Scholar 

  16. Bouhassira D, Attal N, Alchaar H, Boureau F, Brochet B, Bruxelle J, et al. Comparison of pain syndromes associated with nervous or somatic lesions and development of a new neuropathic pain diagnostic questionnaire (DN4). Pain. 2005;114(1–2):29–36.

    Article  PubMed  Google Scholar 

  17. Chong PST, Cros DP. Technology literature review: quantitative sensory testing. Muscle Nerve. 2004;29(5):734–47.

    Article  PubMed  Google Scholar 

  18. Lauria G, Hsieh ST, Johansson O, Kennedy WR, Leger JM, Mellgren SI, et al. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on the use of skin biopsy in the diagnosis of small fiber neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. Eur J Neurol Off J Eur Fed Neurol Soc. 2010;17(7):903–12, e44–9.

    CAS  Google Scholar 

  19. Sommer C, Lauria G. Skin biopsy in the management of peripheral neuropathy. Lancet Neurol. 2007;6(7):632–42.

    Article  PubMed  Google Scholar 

  20. Devigili G, Tugnoli V, Penza P, Camozzi F, Lombardi R, Melli G, et al. The diagnostic criteria for small fibre neuropathy: from symptoms to neuropathology. Brain J Neurol. 2008;131(Pt 7):1912–25.

    Article  Google Scholar 

  21. Holland NR, Stocks A, Hauer P, Cornblath DR, Griffin JW, McArthur JC. Intraepidermal nerve fiber density in patients with painful sensory neuropathy. Neurology. 1997;48(3):708–11.

    Article  CAS  PubMed  Google Scholar 

  22. Lauria G, Morbin M, Lombardi R, Borgna M, Mazzoleni G, Sghirlanzoni A, et al. Axonal swellings predict the degeneration of epidermal nerve fibers in painful neuropathies. Neurology. 2003;61(5):631–6.

    Article  CAS  PubMed  Google Scholar 

  23. Hoeijmakers JGJ, Merkies ISJ, Gerrits MM, Waxman SG, Faber CG. Genetic aspects of sodium channelopathy in small fiber neuropathy. Clin Genet. 2012;82(4):351–8.

    Article  CAS  PubMed  Google Scholar 

  24. Casanova-Molla J, Morales M, Planas-Rigol E, Bosch A, Calvo M, Grau-Junyent JM, et al. Epidermal Langerhans cells in small fiber neuropathies. Pain. 2012;153(5):982–9.

    Article  PubMed  Google Scholar 

  25. Bakkers M, Faber CG, Hoeijmakers JGJ, Lauria G, Merkies ISJ. Small fibers, large impact: quality of life in small-fiber neuropathy. Muscle Nerve. 2014;49(3):329–36.

    Article  PubMed  Google Scholar 

  26. Ho TW, Backonja M, Ma J, Leibensperger H, Froman S, Polydefkis M. Efficient assessment of neuropathic pain drugs in patients with small fiber sensory neuropathies. Pain. 2009;141(1–2):19–24.

    Article  CAS  PubMed  Google Scholar 

  27. Chiang M-C, Tseng M-T, Pan C-L, Chao C-C, Hsieh S-T. Progress in the treatment of small fiber peripheral neuropathy. Expert Rev Neurother. 2015;15(3):305–13.

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Dermatology, University Hospital, Brest, France

    Emilie Brenaut MD

  2. Laboratory of Neurosciences of Brest, University of Western Brittany, Brest, France

    Emilie Brenaut MD

  3. Laboratory of Neurosciences of Brest, and Department of Dermatology, University Hospital, and University of Brest, Brest, France

    Laurent Misery MD, PhD

  4. Department of Dermatology and Laboratory of Skin Neurophysiology, University of Western Brittany, Brest, France

    Laurent Misery MD, PhD

Authors
  1. Emilie Brenaut MD
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  2. Laurent Misery MD, PhD
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Correspondence to Emilie Brenaut MD .

Editor information

Editors and Affiliations

  1. Department of Dermatology, University of Brest, Brest, France

    Laurent Misery

  2. Center of Chronic Pruritus, University Hospital Münster, Münster, Nordrhein-Westfalen, Germany

    Sonja Ständer

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Brenaut, E., Misery, L. (2016). Small-Fibre Neuropathies. In: Misery, L., Ständer, S. (eds) Pruritus. Springer, Cham. https://doi.org/10.1007/978-3-319-33142-3_32

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  • DOI: https://doi.org/10.1007/978-3-319-33142-3_32

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-33140-9

  • Online ISBN: 978-3-319-33142-3

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