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2.1
Primary Erythromelalgia

Presentation

He denied systemic symptoms and he did not present any other comorbidities or family history. [scielo.br]

The authors present the case of a white, 33-years-old woman, with predominantly involved hand. [pesquisa.bvsalud.org]

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]

Entire Body System

  • 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]

Workup

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]

Treatment

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 with aspirin and nifedipine was prescribed; the clinical progress of the patient was successful (AU). [pesquisa.bvsalud.org]

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]

Methods: 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. [eurekaselect.com]

Prognosis

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]

Etiology

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

Through a detailed interview and complementary tests they concluded that the etiological variant presented by the patient was a primary or idiopathic erythromelalgia. [pesquisa.bvsalud.org]

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]

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]

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]

Epidemiology

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]

Pathophysiology

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]

Prevention

Prevention - Erythromelalgia Not supplied. [checkorphan.org]

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]

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]

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