A case of sporadic erythromelalgia presenting with small fibre neuropathy

Patel, Pavan; Zhang, Yan; Unikel, Lynne H; Edwards, Christopher

doi:10.1136/bcr-2019-230549

Cited by 3 publications

(6 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Most of the selected articles for the review were used skin biopsy for the definitive diagnosis and/or genetic tests [ 54 , 57 ] ( Table 1 ). The skin biopsy was used alone [ 32 , 48 , 62 ] or in the most cases together with other diagnostic procedure, as nerve conduction examinations [ 33 , 41 , 56 ] or scales to assess the severity of the neuropathic symptoms ( Table 1 ).…”

Section: Resultsmentioning

confidence: 99%

A Systematic Review of the Diagnostic Methods of Small Fiber Neuropathies in Rehabilitation

2020

View full text Add to dashboard Cite

This systematic review describes the several methods to diagnose and measure the severity of small fiber neuropathies and aims to guide the physician to define all the diagnostic approaches for adopting the best strategies described in the current literature. The search was conducted in PubMed, EMBASE, Cochrane Library and Web of Science. Two reviewers independently reviewed and came to consensus on which articles met inclusion/exclusion criteria. The authors excluded all the duplicates, animals’ studies, and included the English articles in which the diagnostic measures were finalized to assess the effectiveness of rehabilitation and pharmacologic treatment of patients with small fiber neuropathies. The search identified a total of 975 articles with the keywords “small fiber neuropathy” AND “rehabilitation” OR “therapy” OR “treatment”. Seventy-eight selected full-text were analyzed by the reviewers. Forty-one publications met the inclusion criteria and were included in the systematic review. Despite the range of diagnostic tools for the assessment of small fiber neuropathy, other robust trials are needed. In addition, always different diagnostic approaches are used, a unique protocol could be important for the clinicians. More research is needed to build evidence for the best diagnostic methodologies and to delineate a definitive diagnostic protocol.

show abstract

Section: Resultsmentioning

confidence: 99%

A Systematic Review of the Diagnostic Methods of Small Fiber Neuropathies in Rehabilitation

2020

View full text Add to dashboard Cite

show abstract

“…56 In a single patient with erythromelalgia and SFN manifesting with painful flushing and burning paresthesias of the proximal extremities, 400 mg/day carbamazepine significantly reduced pain in the 31-year-old patient. 57 During a one-year follow-up not only pain had almost completely resolved but also dermal flare-ups. 57 In a single patient with non-specified SFN application of lacosamide resulted in a decline of the VAS from 7.5 to 1.5.…”

Section: Painmentioning

confidence: 99%

“…57 During a one-year follow-up not only pain had almost completely resolved but also dermal flare-ups. 57 In a single patient with non-specified SFN application of lacosamide resulted in a decline of the VAS from 7.5 to 1.5. 58 Microneurography documented reduced spontaneous nociceptor activity.…”

Section: Painmentioning

confidence: 99%

Small fiber neuropathy

Finsterer

Scorza

2022

Acta Neuro Scandinavica

View full text Add to dashboard Cite

Small fiber neuropathy (SFN) is a peripheral nervous system disease due to affection of A‐delta or C‐fibers in a proximal, distal, or diffuse distribution. Selective SFN (without large fiber affection) manifests with pain, sensory disturbances, or autonomic dysfunction. Though uniform diagnostic criteria are unavailable, most of them request typical clinical features and reduced intra‐epidermal nerve fiber density on proximal or distal skin biopsy. Little consensus has been reached about the treatment of SFN, why this narrative review aims at summarizing and discussing treatment options for SFN. Treatment of SFN can be classified as symptomatic, pathophysiologic, or causal. Prerequisites for treating SFN are an established diagnosis, knowledge about the symptoms and signs, and the etiology. Pain usually responds to oral/intravenous pain killers, antidepressants, anti‐seizure drugs, or topical, transdermal specifications. Some of the autonomic disturbances respond favorably to symptomatic treatment. SFN related to Fabry disease or hATTR are accessible to pathogenesis‐related therapy. Immune‐mediated SFN responds to immunosuppression or immune‐modulation. Several of the secondary SFNs respond to causal treatment of the underlying disorder. In conclusion, treatment of SFN relies on a multimodal concept and includes causative, pathophysiologic, and symptomatic measures. It strongly depends on the clinical presentation, diagnosis, and etiology, why it is crucial before initiation of treatment to fix the diagnosis and etiology. Due to the heterogeneous clinical presentation and multi‐causality, treatment of SFN should be individualized with the goal of controlling the underlying cause, alleviating pain, and optimizing functionality.

show abstract

“…Many different treatments were experienced. Opioid analgesics [8,21,24] or nonopioid analgesic [22,26], corticosteroids [8,10,33,34,37,44], intravenous immunoglobulin (IVIG) alone [8,12,15,23,27,31,37,39,46,47] or in combination with other specific drugs, such as azathioprine [29], anti-epileptic drugs [4,11,13,16,18,28,32], immunotherapy [14,19,37], hormone therapy [7,43]. Less used are the following therapeutic strategies, in used for specific disorders, such as ARA290, an erythropoietin derivate for sarcoidosis SFN [45], recombinant human nerve growth factor for diabetic SFN [5], propranolol for SFN related to aquagenic pruritus [9], plasma exchange therapy for complex regional pain syndrome [6], enzyme replacement therapy for Fabry related SFN [17,35], botulinum toxin type A for keloid [38].…”

Section: Pharmacologic and Rehabilitation Therapymentioning

confidence: 99%

“…De Greef et al [11,13], Namer et al [28] and Brouwer et al [60] assessed the efficacy, safety, and tolerability of lacosamide, an anticonvulsant, in patients with SCN-associated small fiber neuropathy. Carbamazepine is useful to reduce SFN-related neuropathy [32] too. Gabapentin and naproxen [4] or duloxetine [18] were used for SFN associated with hantavirus infection [4] or in the absence of results with other therapies [4].…”

Section: The Pharmacological Approachesmentioning

confidence: 99%

A Systematic Review of Pharmacologic and Rehabilitative Treatment of Small Fiber Neuropathies

et al. 2020

View full text Add to dashboard Cite

The aim of this systematic review is to guide the physician in defining the pharmacologic and rehabilitative therapeutic approaches for adopting the best strategies described in the current literature. The search was conducted in PubMed, EMBASE, Cochrane Library and Web of Science to identify the treatment of small fiber neuropathies. Two reviewers independently reviewed and came to a consensus on which articles met inclusion/exclusion criteria. The authors excluded the duplicates, animal studies and included the English articles in which the treatment of patients with small fiber neuropathies was described. The search identified a total of 975 articles with the keywords “small fiber neuropathy” AND “rehabilitation” OR “therapy” OR “treatment”. Seventy-eight selected full-text were analyzed by the reviewers. Forty-two publications met the inclusion criteria and were included in the systematic review to describe the rehabilitative and pharmacologic treatment of small fiber neuropathies. Despite the range of different protocols of treatment for small fiber neuropathy, other robust trials are needed. In addition, always different therapeutic approaches are used; a unique protocol could be important for the clinicians. More research is needed to build evidence for the best strategy and to delineate a definitive therapeutic protocol.

show abstract

A case of sporadic erythromelalgia presenting with small fibre neuropathy

Cited by 3 publications

References 9 publications

A Systematic Review of the Diagnostic Methods of Small Fiber Neuropathies in Rehabilitation

A Systematic Review of the Diagnostic Methods of Small Fiber Neuropathies in Rehabilitation

Small fiber neuropathy

A Systematic Review of Pharmacologic and Rehabilitative Treatment of Small Fiber Neuropathies

Contact Info

Product

Resources

About