Peripheral neuropathy and livedoid vasculopathy

• Published in: Journal of neurology Vol. 269; no. 7; pp. 3779 - 3788
• Main Authors: Soulages, Antoine, Maisonobe, Thierry, Auzou, Pascal, Petit, Antoine, Allenbach, Yves, Barète, Stéphane, Skopinski, Sophie, Ribeiro, Emmanuel, Jullié, Marie-Laure, Lamant, Laurence, Brevet, Françoise, Soulages, Xavier, Vallat, Jean-Michel, Martin-Négrier, Marie-Laure, Solé, Guilhem, Duval, Fanny, Carla, Louis, Le Masson, Gwendal, Mathis, Stéphane
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.07.2022; Springer Nature B.V; Springer Verlag
• Subjects: Dermis; Etiology; Ischemia; Life Sciences; Limbs; Literature reviews; Medicine; Medicine & Public Health; Nerves; Neurology; Neuroradiology; Neurosciences; Original Communication; Peripheral nervous system; Peripheral neuropathy; Skin diseases; Skin lesions; Thrombosis; Vascular diseases; Vascular occlusion; Vasculitis; Mononeuropathy; Vasculopathy; Livedoid; Vasculitis; Peripheral neuropathy
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-022-11007-z

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.