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Peripheral neuropathy and livedoid vasculopathy

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Abstract

Background

Livedoid vasculopathy (LV) is a chronic dermatosis associated with micro-thrombosis of the vessels of the dermis, leading to ischemic lesions and painful skin ulcerations of the lower limbs. This thrombosing occlusive vasculopathy, clearly distinct from ‘classical vasculitis’ (not related to alteration of vessel walls), may lead to peripheral neuropathy.

Objective

To clarify the main clinical, electrophysiological and pathological characteristics of peripheral neuropathy linked to LV.

Method

We presented a series of personal cases of peripheral neuropathy due to LV. We also conducted a review of the literature (since the first description of LV in 1974) using multiple combinations of keywords from ‘PubMed’, ‘Google Scholar’ and ‘ScienceDirect’ databases according to the ‘Preferred Reporting Items for Systematic reviews and Meta-Analyses’ guidelines.

Results

We identified 16 patients (6 personal cases and 10 cases from the medical literature). Our personal cases were five females and one male, with a median age (at the onset of cutaneous signs of LV) of 38 (range 25–62). Several types of skin lesions of the lower limbs were observed. Median age at the onset of peripheral neuropathy symptoms was 48 years (range 29–66), with a main clinical and electrophysiological pattern of mononeuropathy multiplex.

Discussion

We observed a typical pattern of peripheral neuropathy, mostly mononeuropathy multiplex, whose pathophysiology might be related to occlusions of the small vessels of the nerves, as seen in the dermis. Moreover, LV may also be associated with other types of peripheral neuropathies (sometimes of autoimmune etiology) not directly related to the skin lesions.

Conclusion

The ‘ischemic form’ of peripheral neuropathy linked to LV is mainly responsible for sensory disturbances (with multifocal distribution), sometimes for motor disturbances. This type of peripheral neuropathy has to be distinguished from ‘classical vasculitic neuropathies’ which are usually treated with antithrombotic therapies.

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Abbreviations

CIDP:

Chronic inflammatory demyelinating polyradiculoneuropathy

cPAN:

Cutaneous polyarteritis nodose

HES:

Hematoxylin–eosin-saffron

Ig:

Immunoglobulin

IVIg:

Intravenous immunoglobulin

LV:

Livedoid vasculopathy

LV-PN:

Peripheral neuropathy due to livedoid vasculopathy

MF:

Myelinated fibers

MM:

Mononeuropathy multiplex

MTHFR:

Methylenetetrahydrofolate reductase

NB:

Nerve biopsy

PAN:

Polyarteritis nodosa

PAS:

Periodic Acid-Schiff

PN:

Peripheral neuropathy

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Funding

This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Author information

Authors and Affiliations

  1. Department of Neurology, Nerve-Muscle Unit, Referral Center for Neuromuscular Diseases AOC, University Hospital of Bordeaux (CHU Bordeaux, Pellegrin Hospital), Bordeaux, France

    Antoine Soulages, Guilhem Solé, Fanny Duval, Louis Carla, Gwendal Le Masson & Stéphane Mathis

  2. Department of Clinical Neurophysiology, AP-HP, Pitié-Salpêtrière Hospital, Paris, France

    Thierry Maisonobe

  3. Department of Neurology, CHR Orléans, Orléans, France

    Pascal Auzou

  4. Department of Dermatology, AP-HP, Saint-Louis Hospital, Paris, France

    Antoine Petit

  5. Department of Internal Medicine and Clinical Immunology, AP-HP, Pitié-Salpêtrière Hospital, Paris, France

    Yves Allenbach

  6. Department of Dermatology, Sorbonne Université (UPMC Paris-6), Paris, France

    Stéphane Barète

  7. Department of Vascular Medicine, University Hospital of Bordeaux (CHU Bordeaux, Saint-André Hospital), Bordeaux, France

    Sophie Skopinski

  8. Department of Internal Medicine and Tropical Diseases, University Hospital of Bordeaux (CHU Bordeaux, Saint-André Hospital), Bordeaux, France

    Emmanuel Ribeiro

  9. Department of Pathology, University Hospital of Bordeaux (CHU Bordeaux, Haut-Lévêque Hospital), Pessac, France

    Marie-Laure Jullié

  10. Department of Pathology, Institut Universitaire du Cancer de Toulouse, Oncopole (IUC-T), Toulouse, France

    Laurence Lamant

  11. Department of Internal Medicine, Rodez Hospital, Rodez, France

    Françoise Brevet

  12. Neurology Office, 23 Boulevard de la République, Rodez, France

    Xavier Soulages

  13. Department of Neurology, University Hospital of Limoges (Dupuytren Hospital), Limoges, France

    Jean-Michel Vallat

  14. Department of Pathology, University Hospital of Bordeaux (CHU Bordeaux, Pellegrin Hospital), Bordeaux, France

    Marie-Laure Martin-Négrier

Authors
  1. Antoine Soulages
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  2. Thierry Maisonobe
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  3. Pascal Auzou
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  4. Antoine Petit
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  5. Yves Allenbach
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  7. Sophie Skopinski
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  8. Emmanuel Ribeiro
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  9. Marie-Laure Jullié
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  10. Laurence Lamant
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  11. Françoise Brevet
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  12. Xavier Soulages
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  13. Jean-Michel Vallat
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  14. Marie-Laure Martin-Négrier
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  15. Guilhem Solé
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  16. Fanny Duval
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  17. Louis Carla
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  18. Gwendal Le Masson
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  19. Stéphane Mathis
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Corresponding author

Correspondence to Stéphane Mathis.

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The authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript.

Ethical standards

This study was in compliance with the ethical principles of the conference of Helsinki.

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Soulages, A., Maisonobe, T., Auzou, P. et al. Peripheral neuropathy and livedoid vasculopathy. J Neurol 269, 3779–3788 (2022). https://doi.org/10.1007/s00415-022-11007-z

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  • DOI: https://doi.org/10.1007/s00415-022-11007-z

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