Expanding the spectrum of livedoid vasculopathy: peculiar neuromuscular manifestations

Allenbach, Yves; Tourte, M.; Stenzel, Werner; Goebel, Hans‐Hilmar; Maisonobe, Tierry; Françès, Camille; Barète, Stéphane; Benveniste, Olivier

doi:10.1111/nan.12243

Cited by 14 publications

(18 citation statements)

References 8 publications

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“…www.medicaljournals.se/acta (29). Peripheral neuropathy has been rarely described with a total of 9 detailed cases in the literature: one sensory ganglionopathy, one axonal neuropathy and 7 cases of mononeuritis multiplex (8,(10)(11)(12)(13)(14)(15).…”

Section: Discussionmentioning

confidence: 99%

Livedoid Vasculopathy: A French Observational Study Including Therapeutic Options

Gardette¹,

Moguelet²,

Bouaziz³

et al. 2018

Acta Derm Venerol

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Livedoid vasculopathy is a rare thrombotic cutaneous disease. This observational study aimed to assess the clinical and biological features of livedoid vasculopathy and the efficacy of treatments. Patients enrolled had typical livedoid vasculopathy both clinically and histologically. Investigation of thrombophilia was performed. Electromyography was undertaken in the presence of symptoms suggesting peripheral neuropathy. Eighteen women and 8 men were included, with a mean age of 35.5 years at onset. Twenty patients had at least one thrombophilia factor. Ten patients had a peripheral neuropathy with 2 of these patients demonstrating a specific thrombo-occlusive vasculopathy on muscle biopsy. Anticoagulation with low molecular weight heparin was the most prescribed therapy and was associated with the best outcome (effective in 14 patients). Eight patients had severe disease refractory to anticoagulation and required intravenous immunoglobulins, producing a good response in 6 patients.

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Section: Discussionmentioning

confidence: 99%

Livedoid Vasculopathy: A French Observational Study Including Therapeutic Options

Gardette¹,

Moguelet²,

Bouaziz³

et al. 2018

Acta Derm Venerol

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“…The causal role of a primary inflammatory vasculopathy at the capillary level was, however, challenged recently, arguing that complement deposition in capillaries is not consistently present in DM, whereas necrotizing myopathy with pipestem capillaries is not associated with PMA despite a very prominent complement deposition [ 8 ]. PMA is also not observed in livedoid vasculopathy which also features dramatic capillary dropout and perimysial inflammation in muscle tissue [ 1 ]. In summary, inflammation per se and capillary dropout may also not fully explain DM-specific PMA.…”

Section: Discussionmentioning

confidence: 99%

Differential roles of hypoxia and innate immunity in juvenile and adult dermatomyositis

Preuße

Allenbach

Hoffmann

et al. 2016

acta neuropathol commun

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Dermatomyositis (DM) can occur in both adults and juveniles with considerable clinical differences. The links between immune-mediated mechanisms and vasculopathy with respect to development of perifascicular pathology are incompletely understood. We investigated skeletal muscle from newly diagnosed, treatment-naïve juvenile (jDM) and adult dermatomyositis (aDM) patients focusing on hypoxia-related pathomechanisms, vessel pathology, and immune mechanisms especially in the perifascicular region. Therefore, we assessed the skeletal muscle biopsies from 21 aDM, and 15 jDM patients by immunohistochemistry and electron microscopy. Transcriptional analyses of genes involved in hypoxia, as well as in innate and adaptive immunity were performed by quantitative Polymerase chain reaction (qPCR) of whole tissue cross sections including perifascicular muscle fibers.Through these analysis, we found that basic features of DM, like perifascicular atrophy and inflammatory infiltrates, were present at similar levels in jDM and aDM patients. However, jDM was characterized by predominantly hypoxia-driven pathology in perifascicular small fibers and by macrophages expressing markers of hypoxia. A more pronounced regional loss of capillaries, but no relevant activation of type-1 Interferon (IFN)-associated pathways was noted. Conversely, in aDM, IFN-related genes were expressed at significantly elevated levels, and Interferon-stimulated gene (ISG)15 was strongly positive in small perifascicular fibers whereas hypoxia-related mechanisms did not play a significant role.In our study we could provide new molecular data suggesting a conspicuous pathophysiological ‘dichotomy’ between jDM and aDM: In jDM, perifascicular atrophy is tightly linked to hypoxia-related pathology, and poorly to innate immunity. In aDM, perifascicular atrophy is prominently associated with molecules driving innate immunity, while hypoxia-related mechanisms seem to be less relevant.Electronic supplementary materialThe online version of this article (doi:10.1186/s40478-016-0308-5) contains supplementary material, which is available to authorized users.

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“…A clue regarding this mechanism can be found in the case of livedoid vasculopathy, a very rare condition characterized by a thrombo-occlusive vasculopathy of superficial dermal micro-vessels (purpura, livedo and ulcerations) due to thrombophilia. This condition may also affect the muscle when it causes a severe vasculopathy leading to perifascicular myofiber damage [100] . This observation suggests that the perifascicular region is especially sensitive to ischemic insult.…”

Section: Pathophysiology Of Dmmentioning

confidence: 99%

239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14–16 December 2018

Mammen¹,

Allenbach

Stenzel

et al. 2020

Neuromuscular Disorders

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Expanding the spectrum of livedoid vasculopathy: peculiar neuromuscular manifestations

Cited by 14 publications

References 8 publications

Livedoid Vasculopathy: A French Observational Study Including Therapeutic Options

Livedoid Vasculopathy: A French Observational Study Including Therapeutic Options

Differential roles of hypoxia and innate immunity in juvenile and adult dermatomyositis

239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14–16 December 2018

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