Acute monophasic erythromelalgia pain in five children diagnosed as small-fiber neuropathy

Faignart, Nicole; Nguyen, Karine; Soroken, Cindy; Poloni, Claudia; Downs, Heather; Laubscher, Bernard; Korff, Christian; Oaklander, Anne Louise; Perez, Eliane Roulet

doi:10.1016/j.ejpn.2020.06.004

Cited by 13 publications

(14 citation statements)

References 58 publications

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“… 8 Although the pain manifestations are similar in NeP and SFN cohorts, the antibody-positive NeP cases showed a young age at onset and female preponderance, reflecting the features of background diseases. Given that childhood-onset SFN is not rare and is often caused by dysimmunity and improved by immunotherapy, 2 , 24 , 25 plexin D1-IgG should be examined in childhood-onset SFN in future studies.…”

Section: Discussionmentioning

confidence: 99%

Antiplexin D1 Antibodies Relate to Small Fiber Neuropathy and Induce Neuropathic Pain in Animals

Fujii

Lee

Miyachi

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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ObjectivesTo assess the prevalence of antiplexin D1 antibodies (plexin D1-immunoglobulin G [IgG]) in small fiber neuropathy (SFN) and the effects of these antibodies in vivo.MethodsWe developed an ELISA for plexin D1-IgG using a recombinant extracellular domain of human plexin D1 containing the major epitope and sera from 58 subjects previously studied with a standard tissue-based indirect immunofluorescence assay (TBA). We screened 63 patients with probable SFN and 55 healthy controls (HCs) for serum plexin D1-IgG using ELISA. The results were confirmed by TBA. IgG from 3 plexin D1-IgG-positive patients, 2 plexin D1-IgG-negative inflammatory disease controls, and 2 HCs was intrathecally injected into mice, which were assessed for mechanical and thermal hypersensitivity 24 and 48 hours after injection.ResultsThe ELISA had 75% sensitivity and 100% specificity using the TBA as a standard, and the coincidence rate of ELISA to TBA was 96.6% (56/58). The frequency of plexin D1-IgG was higher in patients with SFN than in HCs (12.7% [8/63] vs 0.0% [0/55], p = 0.007). Purified IgG from all 3 plexin D1-IgG-positive patients, but not 2 plexin D1-IgG-negative patients, induced significant mechanical and/or thermal hypersensitivity compared with IgG from HCs. In mice injected with plexin D1-IgG-positive but not D1-IgG-negative patient IgG, phosphorylated extracellular signal-regulated protein kinase immunoreactivity, an activation marker, was confined to small dorsal root ganglion neurons and was significantly more abundant than in mice injected with HC IgG.ConclusionsPlexin D1-IgG is pathogenic but with low prevalence and is a potential biomarker for immunotherapy in SFN.

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Section: Discussionmentioning

confidence: 99%

Antiplexin D1 Antibodies Relate to Small Fiber Neuropathy and Induce Neuropathic Pain in Animals

Fujii

Lee

Miyachi

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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“… 24 In children, immune-mediated and inflammatory SFN seem to be amenable to disease-modifying pharmacologic therapy including IVIg and corticosteroids. 9 , 31 IVIg, however, has not been found to be useful in the cases of idiopathic SFN in adults. 32 …”

Section: Small Fiber Neuropathymentioning

confidence: 98%

“… 20 SFN as a disorder has only been characterized in pediatric patients recently; although there is evidence that this condition exists among school-aged children, epidemiologic data and extensive literature pertaining to this population are unavailable. 9 , 10 As such, the literature reviewed pertaining to SFN largely describes the condition in adults.…”

Section: Small Fiber Neuropathymentioning

confidence: 99%

“… 11 This technique has been used to diagnose SFN in several pediatric patients, with age-adjusted norms; however, no protocol has been established for diagnosis of SFN in this population. 9 , 10 , 28 …”

Section: Small Fiber Neuropathymentioning

confidence: 99%

“…SFN as a disorder has only been characterized in pediatric patients quite recently; epidemiologic data is thus unavailable; however, there is evidence that this condition exists among school-aged children with widespread pain; some of these patients had previously been diagnosed with fibromyalgia and others with phenomena including acute monophasic erythromelalgia, central sensitization, pain-amplification syndrome and chronic fatigue. 9 , 10 …”

Section: Introductionmentioning

confidence: 99%

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Implications of Nerve Fiber Density on the Diagnosis and Treatment of Juvenile Fibromyalgia

Ahmed¹,

Vigouroux²,

Ingelmo³

2022

JPR

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Juvenile fibromyalgia (JFM) is a condition that presents as chronic widespread musculoskeletal pain and affects children and adolescents. JFM remains a challenging diagnosis, as it is both based on subjective criteria and the pathogenesis is poorly understood. Small fiber neuropathy (SFN) is a distinct condition, which is characterized by pathology of small A-delta and C fibers, and can present similarly to JFM. Small fiber pathology is characterized by reduced intraepidermal nerve fiber density (IENFD) on skin biopsy. Recent studies have found that as many as half of patients with JFM can demonstrate decreased IENFD, in pattern similar to SFN. This phenomenon has been referred to as small fiber pathology. The meaning of these findings was disputed; however, the current consensus remains that fibromyalgia and SFN are distinct conditions. Additionally, among patients with fibromyalgia, there are two phenotypes: those with small fiber pathology and those without. The purpose of this review was to characterize the role assessment of IENFD plays in the clinical context. We conducted a narrative review of pertinent articles pertaining to JFM, SFN and small fiber pathology in fibromyalgia. We concluded that assessment of IENFD should be completed if SFN is suspected either when a patient first presents or in patients who were previously diagnosed with fibromyalgia and SFN is later suspected. Distinguishing between JFM and SFN is important because recommended therapies differ between the two conditions. However, there is no evidence to support the use of skin biopsy to distinguish between the two discussed fibromyalgia phenotypes. More studies are needed to elucidate whether IENFD varies with morbidity and if both fibromyalgia phenotypes vary in their response to different therapeutic regimens.

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Erythromelalgie

Bauerschmitz¹

2023

Klinische Angiologie

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Acute monophasic erythromelalgia pain in five children diagnosed as small-fiber neuropathy

Cited by 13 publications

References 58 publications

Antiplexin D1 Antibodies Relate to Small Fiber Neuropathy and Induce Neuropathic Pain in Animals

Antiplexin D1 Antibodies Relate to Small Fiber Neuropathy and Induce Neuropathic Pain in Animals

Implications of Nerve Fiber Density on the Diagnosis and Treatment of Juvenile Fibromyalgia

Erythromelalgie

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