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LETTER TO THE EDITOR
Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 83-84

Red, hot, painful feet and legs: Erythromelalgia


Department of DVL, GSL Medical College, Rajahmundry, Andhra Pradesh, India

Date of Web Publication22-Mar-2018

Correspondence Address:
Dr. Raghurama R Gandikota
Department of DVL, GSL Medical College, Rajahmundry - 533 296, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_7_18

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How to cite this article:
Kollipara H, Gandikota RR, Arumilli KP, Priyanka S. Red, hot, painful feet and legs: Erythromelalgia. J NTR Univ Health Sci 2018;7:83-4

How to cite this URL:
Kollipara H, Gandikota RR, Arumilli KP, Priyanka S. Red, hot, painful feet and legs: Erythromelalgia. J NTR Univ Health Sci [serial online] 2018 [cited 2020 Sep 25];7:83-4. Available from: http://www.jdrntruhs.org/text.asp?2018/7/1/83/228152



Sir,

Erythromelalgia (EM), a rare neurovascular disease characterized by episodic attacks of severe burning pain, erythema, and warmth, typically affects the feet, limbs, and hands. It is a disabling disorder which may be primary or secondary. The first reported case was by Graves in 1834 and Mitchell suggested the term EM in 1878, based on cardinal symptoms and signs: erythus (red), melos (extremities), and algos (pain). The incidence is found to be less than 2 in 100,000 persons per year.[1]

A 25-year-old otherwise healthy woman presented with complaints of episodic pain of both lower limbs and feet for 10 years and erythema of 3 years. No other family member was suffering from similar complaints. A detailed clinical history has revealed aggravation of pain and erythema whenever she exposes to hot and humid climate. For relief she used to spend most of her time in air-conditioned rooms and cool places. Despite various consultations by rheumatologists, neurologists, and physicians and usage of systemic steroids, methotrexate, mycophenolate mofetil, and analgesics for all these years, her clinical condition was not diagnosed. On physical examination, her both lower limbs including feet were swollen, red, and tender [Figure 1]a and [Figure 1]b. Upper extremities were normal. There were no other cutaneous lesions. Peripheral nerves were normal and not thickened. There was no sensory impairment on the limbs. Peripheral pulses were normally felt. No regional lymphadenopathy was present. All other systems were normal. All hematological, biochemical, serological, and connective tissue disease profile tests were normal. Nerve conduction studies and autonomic nerve function tests were normal. Histopathological examination of skin biopsy specimen was unremarkable, except for mild perivascular lymphocytic infiltrate in the dermis. A final diagnosis of primary EM was made on the basis of clinical history, examination, and investigations. Patient was counseled about the disease and its chronic course. She was advised to avoid precipitating factors such as increased ambient heat, exercise, and maintain normal lifestyle. She was also advised to expose affected areas to cool water for short durations like 5–10 minutes every 1–2 hours or use fan for short periods of time and limb elevation. Initially she was given aspirin 150 mg b.d and nortryptiline 25 mg daily for 8 weeks. There was not much improvement. Aspirin was stopped and instead gabentine 300 mg twice daily was started. She is under regular follow-up. Patient consent was obtained before sending this article for publication.
Figure 1: (a) Erythematous swollen feet and legs, (b) Erythematous, swollen feet

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EM is a chronic, debilitating, functional neurovascular disease characterized by paroxysmal pain and erythema of extremities.[2] EM is more common in females and adults, but can also occur in children. Exacerbating factors include heat, exercise, emotional stress, injury, and pressure. In severe cases, patients perform ice water immersion constantly that lead to maceration of skin, nonhealing ulcers, infection, necrosis, and amputation.[1],[3],[4] EM can be primary or secondary. Primary begins spontaneously at any age. It may occur sporadically but when familial, it is inherited in autosomal dominant manner. A number of hypotheses have been put forward to explain primary EM, including vascular shunting, neuropathic, microvascular, and inflammatory etiologies. Familial form of EM is due to a genetic mutation in gene SCN9A on chromosome 2q which encodes voltage-gated sodium channels expressed in nociceptive dorsal root ganglion (DRG) and sympathetic ganglion neurons. This leads to hyperexcitability of C fibers in DRG and causes increased burning pain and blood flow to the skin.[5] Secondary EM is associated with myeloproliferative disorders (polycythemia, thrombocytopenia), neuropathies, hypertension, and autoimmune diseases. Other causes include drugs (calcium channel blockers, misoprostone, bromocriptine), mushroom and mercury poisoning, certain pox virus infections (EM-related pox viruses), and diabetes mellitus. Living with EM can result in deterioration of quality of life.[1],[5] Recently, corticosteroid responsive subset of EM patients was identified and corticosteroid intervention in early stages of disease is useful.[6] EM is a diagnosis often made on clinical history and may be confused with Raynaud's disease, Raynaud's phenomenon, vasculitis, thromboanginitis obliterans, complex regional pain syndrome, and Fabry's disease.[1]

Treatment options largely consist of lifestyle adjustment and pain management. A variety of topical and systemic therapies have been used for EM. Topical measures include capsaicin, lidocaine, diclofenac, and gabentine patches. Various systemic drugs include calcium antagonists, magnesium selective serotonin reuptake inhibitors, aspirin, tricyclic-antidepressants, gabapentin, carbamazepine, antihistamines, clonazepam, misoprostol, cyproheptadine, and β-blockers. Rarely epidural analgesia or bilateral sympathectomies are used for EM. But, efficacy of data are limited and inconclusive. Prognostic data of EM are limited.[1]

As in our case, majority of these individuals go unrecognized despite seeking medical help. Considering the disabling nature of EM, knowledge and awareness of this rare clinical entity is essential for early diagnosis and counseling.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Jay S. Cohen, Erythromelalgia: New theories and new therapies. J Am Acad Dermatol 2000;43:841-7.  Back to cited text no. 1
    
2.
Davis MD, Sandroni P, Rooke TW, Low PA. Erythromelalgia: Vasculopathy, neuropathy, or both? A prospective study of vascular and neurophysiologic studies in erythromelalgia. Arch Dermatol 2006;142:1583-8.  Back to cited text no. 2
    
3.
Damodar SS, Smitha P, Nirmal B, Sudhir NU, Ballambat PS. Hansen's disease associated with erythromelalgia mimicking Lupus erythematosus. Indian Dermatol Online J 2014;5:59-62.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Krishnan SG, Yesudian DP, Jayaraman M, Janaki VR, Raj BJ. Erythromelalgia responding to aspirin. Indian J Dermatol Venereol Leprol 1996;62:204-5.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Friberg D, Chen T, Tarr G, van Rij A. Erythromelalgia? A clinical study of people who experience red, hot, painful feet in the community. Int J Vasc Med 2013;2013:864961.  Back to cited text no. 5
    
6.
Pagani-Estévez GL, Sandroni P, Davis MD, Watson JC. Erythromelalgia: Identification of a corticosteroid-responsive subset. J Am Acad Dermatol 2017;76:506-11.  Back to cited text no. 6
    


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