We present two cases of primary erythromelalgia both of whom presented with a history of several months of severe burning pain in both hands and feet. Both patients had received multiple pain medications with no improvement in symptoms. [ncbi.nlm.nih.gov]

• Camping

  He returned to and completed school, attended summer camp, and enjoys an active happy life. He walks without precipitating pain in his feet and sleeps 9 to 10 hours every night. [ncbi.nlm.nih.gov]

• Collapse

  Lidocaine toxicity can result in neurotoxicity, with central nervous system depression or seizures, and cardiovascular toxicity, with hypotension, arrhythmias, and cardiovascular collapse. [pediatrics.aappublications.org]

  Unfortunately, neurogenic shock with collapse took place due to severe pain after debridement surgery of her feet. Genetic Analysis DNA was extracted from patients’ blood using Genomic DNA Extraction Kit (Geneaid, Taipei, Taiwan). [journals.plos.org]

  Because of the lack of obvious neurological signs, patients may have seen multiple health professionals and been given a number diagnoses, including ‘plantar fasciitis’ or ‘collapsed arches’, in some cases leading to unnecessary surgery (which exacerbates [pn.bmj.com]

• Recurrent Infection

  The implants were removed twice because of recurrent infection. Finally, the patient was treated with bilateral electrical stimulation of the ventral posterolateral thalamic nucleus, which resulted in important pain control until 3 years later. [ncbi.nlm.nih.gov]

• Hyperalgesia

  When comparing the individual results, there is a tendency to clustering of patients in two separate groups; reduced small fiber input/no hyperalgesia and normal thermal thresholds/hyperalgesia. [ncbi.nlm.nih.gov]

  When comparing the individual results, there is a tendency to clustering of patients in two separate groups; reduced small fiber input/no hyperalgesia and normal thermal thresholds/ hyperalgesia. [journals.lww.com]

  This resulted in complete resolution of both allodynia and hyperalgesia. Dosage was increased to 1.8 mcg/die. [painmatters.wordpress.com]

  Furthermore, the hyperalgesia and allodynia had nearly disappeared, allowing the patient to walk unassisted while wearing socks and shoes. [spandidos-publications.com]

  Double and triple spikes may contribute to hyperalgesia in patients with neuropathic pain. [pn.bmj.com]

• Indifference to Pain

  In addition to PE, mutations in SCN9A gene can also result in paroxysmal extreme pain disorder (PEPD; OMIM 167400) [12] and congenital insensitivity (or indifference) to pain (CIP; OMIM 243000) [13]. [journals.plos.org]

Extensive workup was consistent with adult-onset primary erythromelalgia (EM). [dovepress.com]

A rheumatological workup may also be performed. A neurological examination will look for evidence of peripheral neuropathy. [drpribut.com]

On our evaluation, we conducted an extensive rheumatologic workup. This included getting bilateral feet, ankle, and tibia/fibula x rays, which were normal. [omicsonline.org]

A Chinese Han family with typical symptoms of PE was collected and the proband's response to treatment was recorded. All the exons and flanking intronic sequences of SCN9A were amplified with PCR and sequenced. [ncbi.nlm.nih.gov]

Treatment is difficult and controlled therapeutic studies offer little to no guidance. We report on a 12-year-old boy and his first occurrence of primary erythromelalgia. [unboundmedicine.com]

Prognosis Prognosis depends on the severity of the disease. In severe cases the burning pain leads to an urgent need to cool the affected extremities. [orpha.net]

Topical treatment with capsaicin cream SSRI drugs Tricyclic antidepressants eg amitriptyline Gabapentin Step 4: other treatments occasionally used Prostaglandins - misoprostol Intravenous infusions of nitroprusside, or prostaglandin Prognosis The prognosis [pcds.org.uk]

Diagnosis - Erythromelalgia Prognosis - Erythromelalgia Avoiding triggers is key to the management of current symptoms, but little is known regarding how this affects the long-term course of an individual's condition. [checkorphan.org]

We also thank the be corrected, the prognosis or threshold level, plasma States after lung cancer. [chicagocovenant.org]

We propose a genotype-channelopathy-phenotype correlation network underlying PE etiology which could provide guidance for future therapeutics. [ncbi.nlm.nih.gov]

Etiology The gene for autosomal dominant erythermalgia, SCN9a, is located on chromosome 2q. SCN9a is a 26 exon gene encoding the voltage-gated sodium channel alpha subunit Nav1.7. [orpha.net]

Genetic etiology of PE is mutations on SCN9A, the encoding gene of a voltage-gated sodium channel subtype Nav1.7. Diagnosis of PE is made upon clinical manifestations and screening for mutations on SCN9A. [regione.lazio.it]

Management strategy Before beginning a treatment regimen, the patient’s condition should be classified according to whether it is primary or secondary, the etiology (for secondary cases), and its severity. [clinicalgate.com]

The etiology of primary Erythromelalgia involves mutations in the sodium ion channel Nav1.7 that in humans is encoded by the SCN9A gene. [omicsonline.org]

Summary Epidemiology It may occur sporadically or as an inherited disease, but less than 30 kindreds with familial primary erythermalgia have been reported in the literature so far. [orpha.net]

"Epidemiology study of an outbreak of epidemic erythromelalgia. Occupation and Health". 21 : 713–714. Xie F, Ning H, Lei Y, Lei S (2010). "A brief report of an outbreak epidemic erythromelalgia. [en.wikipedia.org]

In one patient with SLE, biopsy results showed vasculitis, and symptoms resolved with immunosuppressive therapy. [21] Reports in Europe and Japan describe cases due to the ingestion of different species of Clitocybe mushrooms. [22, 23] Epidemiology United [emedicine.medscape.com]

Aetiology, epidemiology and pathogenesis The exact incidence and prevalence of SFN is unknown. 1, 11, 12 There have been no satisfactory studies to assess the epidemiology of SFN, because until recently, there was no generally accepted definition for [pn.bmj.com]

[…] mercury, arsenic) Neoplasias: Paraneoplastic syndrome, Colon and breast cancer, Malignant thymoma, Astrocytoma, Subcutaneous panniculitis-like T-cell lymphoma Miscellanea: Influenza and hepatitis vaccine, Amaurosis, Lichen sclerosus, Genetic diseases EPIDEMIOLOGY [scielo.br]

Microneurography was used to clarify the role of skin sympathetic nerve activity in the pathophysiology of primary erythromelalgia. The patient showed normal skin sympathetic nerve activity but no vasoconstriction response. [ncbi.nlm.nih.gov]

Gain a state-of-the-art understanding of the pathophysiology, diagnosis, and management of arterial disease, venous disease, lymph dysfunction, connective tissue disease, vascular disease, and vascular manifestations of systemic disease. [books.google.com]

Learn the best methods for aggressive patient management and disease prevention to ensure minimal risk of further cardiovascular problems. Stay current with ACC/AHA and ECC guidelines and the best ways to implement them in clinical practice. [books.google.com]

Prevention - Erythromelalgia Not supplied. [checkorphan.org]

For people with other forms of neuropathy that cause burning feet, preventing further nerve damage is equally important. Specific conditions and their treatments include: Vitamin deficiency. [webmd.com]

Individuals with secondary erythromelalgia can prevent another episode by treating the underlying condition. To determine whether you have primary or secondary erythromelalgia, consult your physician. [gifootclinic.com]