Antoine Soulages1, Thierry Maisonobe2, Pascal Auzou3, Antoine Petit4, Yves Allenbach5, Stéphane Barète6, Sophie Skopinski7, Emmanuel Ribeiro8, Marie-Laure Jullié9, Laurence Lamant10, Françoise Brevet11, Xavier Soulages12, Jean-Michel Vallat13, Marie-Laure Martin-Négrier14, Guilhem Solé1, Fanny Duval1, Louis Carla1, Gwendal Le Masson1, Stéphane Mathis15. 1. Department of Neurology, Nerve-Muscle Unit, Referral Center for Neuromuscular Diseases AOC, University Hospital of Bordeaux (CHU Bordeaux, Pellegrin Hospital), Bordeaux, France. 2. Department of Clinical Neurophysiology, AP-HP, Pitié-Salpêtrière Hospital, Paris, France. 3. Department of Neurology, CHR Orléans, Orléans, France. 4. Department of Dermatology, AP-HP, Saint-Louis Hospital, Paris, France. 5. Department of Internal Medicine and Clinical Immunology, AP-HP, Pitié-Salpêtrière Hospital, Paris, France. 6. Department of Dermatology, Sorbonne Université (UPMC Paris-6), Paris, France. 7. Department of Vascular Medicine, University Hospital of Bordeaux (CHU Bordeaux, Saint-André Hospital), Bordeaux, France. 8. Department of Internal Medicine and Tropical Diseases, University Hospital of Bordeaux (CHU Bordeaux, Saint-André Hospital), Bordeaux, France. 9. Department of Pathology, University Hospital of Bordeaux (CHU Bordeaux, Haut-Lévêque Hospital), Pessac, France. 10. Department of Pathology, Institut Universitaire du Cancer de Toulouse, Oncopole (IUC-T), Toulouse, France. 11. Department of Internal Medicine, Rodez Hospital, Rodez, France. 12. Neurology Office, 23 Boulevard de la République, Rodez, France. 13. Department of Neurology, University Hospital of Limoges (Dupuytren Hospital), Limoges, France. 14. Department of Pathology, University Hospital of Bordeaux (CHU Bordeaux, Pellegrin Hospital), Bordeaux, France. 15. Department of Neurology, Nerve-Muscle Unit, Referral Center for Neuromuscular Diseases AOC, University Hospital of Bordeaux (CHU Bordeaux, Pellegrin Hospital), Bordeaux, France. stephane.mathis@chu-bordeaux.fr.
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.
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.