Review of primary and secondary erythromelalgia

Mann, Nirmal S.; King, Thomas H.; Murphy, Ruth

doi:10.1111/ced.13891

Cited by 65 publications

(63 citation statements)

References 25 publications

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“…It often triggered by heat and exercise. Primary erythromelalgia occurs as an autosomal dominant mutation in the SCN9A gene which codes for a voltage-gated sodium channel involved in pain perception [20].…”

Section: Discussionmentioning

confidence: 99%

Heater-Associated Erythema Ab Igne: Case Report and Review of Thermal-Related Skin Conditions

Forouzan¹,

Riahi²,

Cohen³

2020

Cureus

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Erythema ab igne is a thermal-associated skin condition that can occur secondary to persistent direct or indirect contact with heat. Historically, erythema ab igne has been linked to fireplace and stove exposures; more recently, it has been associated with heaters, hot water bottles, and laptops. A 48-year-old woman presented for the evaluation of hyperpigmented, reticulated macular lesions on her distal legs. Additional history revealed that she had developed erythema ab igne secondary to the use of a space heater underneath her desk at work. Her skin condition stopped progressing with removal of the causative agent. In addition to erythema ab igne, heatrelated skin conditions include basal cell carcinomas and squamous cell carcinomas, burns, erythromelalgia, subtypes of urticaria, and ultraviolet-associated disorders. Awareness of thermal-associated skin conditions enables the clinician to establish the appropriate diagnosis based on the associated history of the condition, the morphology of the skin lesion, and, if necessary, correlation with the skin biopsy findings of the cutaneous condition.

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Section: Discussionmentioning

confidence: 99%

Heater-Associated Erythema Ab Igne: Case Report and Review of Thermal-Related Skin Conditions

Forouzan¹,

Riahi²,

Cohen³

2020

Cureus

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“…4 Primary erythromelalgia is an autosomal dominant disorder caused by mutations in the SCN9A gene encoding the Nav1.7 sodium channel in peripheral sensory neurons which lowers the pain threshold. 5 Vascular abnormalities also play a role in the pathophysiology of erythromelalgia. The precapillary sphincters are constricted while the arteriovenous shunts are open, creating an imbalance of increased total perfusion yet deficient nutritive perfusion.…”

Section: Discussionmentioning

confidence: 99%

Novel treatment modality in a case of resistant erythromelalgia

2020

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<p>Erythromelalgia presents with redness, pain and warmth of the extremities, which is exacerbated by warming and relieved by cooling. Treatment of pain in erythromelalgia can be difficult, and often polypharmacy is utilized. No single therapy has been found to provide complete relief for any patient. Therefore, a multidisciplinary approach is required to treat pain and its significant negative impact across many domains of functioning. A 21 year old male diagnosed case of primary erythromelalgia since 5 years, presented multiple times with recurrent pain and erythema in bilateral lower limbs. Patient was treated initially with oral antibiotics, tramadol and prednisolone, without relief. Aspirin, amitriptyline, pregabalin, carbamazepine, atenolol were later added which gave him partial relief. Epidural caudal catheter with daily infusion of anesthesia was added along with topical buprenorphine patch when conservative polypharmacy failed to provide complete relief and later unilateral lumbar sympathectomy was done as a last resort. Adding pentoxifylline and compression therapy improved the patient’s condition drastically and complete remission is in progress. The patient required a multidisciplinary approach for pain management and surgical intervention was required due to his refractory nature. However, this also does not give complete relief and thus a combination of both medical and surgical methods is required for best control of symptoms.</p>

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“…Further research is needed for a more complete understanding of this disease and its basis. This will help in terms of its management, prognosis and ultimately, patient quality of life 9 10…”

Section: Discussionmentioning

confidence: 99%

A case of sporadic erythromelalgia presenting with small fibre neuropathy

et al. 2019

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Erythromelalgia (EM) is a rare disorder of small nerve fibres that leads to painful flushing and burning paresthesisas of the distal extremities and is typically associated with heat or physical activity; relief is found using cooling measures. Its effects are often debilitating in the general population, but this patient had an excellent response to specific treatment options and continues to maintain employment, something many individuals suffering from EM are unable to do. His presentation was also unique in that he had isolated, proximal involvement as his condition progressed whereas typically only the distal extremities are affected. Routine electromyography and nerve conduction studies were normal, whereas nerve biopsy demonstrated findings of small fibre neuropathy. Ultimately, his condition was managed with carbamazepine and his symptoms have almost entirely resolved to date.

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Review of primary and secondary erythromelalgia

Cited by 65 publications

References 25 publications

Heater-Associated Erythema Ab Igne: Case Report and Review of Thermal-Related Skin Conditions

Heater-Associated Erythema Ab Igne: Case Report and Review of Thermal-Related Skin Conditions

Novel treatment modality in a case of resistant erythromelalgia

A case of sporadic erythromelalgia presenting with small fibre neuropathy

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