Microvascular imaging of primary erythromelalgia

Hellmann, Marcin; Imbert, B.; Cracowski, Jean‐Luc

doi:10.20452/pamw.14890

Cited by 2 publications

(5 citation statements)

References 2 publications

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“… 3 Although its mechanisms remain unknown yet, neural or vascular abnormalities or even both are considered to play important roles. 3 As patients with EM always have increased warmth and microvascular blood flux 13 as well as obvious angiotelectasis 4 , 5 during the episodes, those suggested that vasodilation might play important roles in the symptoms of EM.…”

Section: Discussionmentioning

confidence: 99%

Auricular erythermalgia showing excellent response to itraconazole: a case report

et al. 2022

Therapeutic Advances in Chronic Disease

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Erythermalgia, a rare painful disorder, is characterized by recurrent pain attacking, warmth, and erythema that mainly involves the distal extremities. Red ear syndrome shares similar clinical features of erythermalgia afflicting the external ear with unilateral/bilateral distribution. The treatments of both diseases are still difficult without controlled therapeutics available up to date. A 12-year-old boy was referred because of 3 years of recurrent attacking of painful erythema and warmth that involved the ears alone, the episodes occurred several times daily with duration of dozens of minutes to hours for each flare. The symptoms could be relieved by cold water and triggered by heat stimuli as well as exciting and movement, and showed mild response to gabapentin, celecoxib, and topical lidocaine compounds in combination, but moderate to blocking injection of botulinum toxin to nervus auricularis magnus. However, systemic itraconazole 200 mg daily resulted in an excellent response after 5-week treatment, leading to milder erythema, warmth and burning sensation, shorter duration, and fewer relapses. The treatment continued for 6 months and then itraconazole was decreased to 100 mg daily for another 6 months until it was stopped, with maintenance of good conditions. In 3 months of follow-up after the treatment ceased, the patient had only 7 to 8 attacks over 10 days presenting as tolerable erythema that lasted for less than 10 min and relieved spontaneously, with absence of warmth and no need of treatment. We considered the patient to be a variant of erythermalgia rather than a red ear syndrome. The results showed that erythermalgia might involve the ears alone and itraconazole might be a potential agent for its treatment.

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Section: Discussionmentioning

confidence: 99%

Auricular erythermalgia showing excellent response to itraconazole: a case report

et al. 2022

Therapeutic Advances in Chronic Disease

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“…[1,4]. In 2019, 14 EM cases have been reported in the literature despite the high probability of missed diagnosis and non-reported cases [5][6][7][8][9][10][11][12][13][14][15][16][17].…”

Section: Discussionmentioning

confidence: 99%

“…"Primary" erythromelalgia occurs in the absence of its known associations. Patients may have co-morbidities such as hypertension, atrial fibrillation, diabetes, Raynaud's phenomenon, peripheral neuropathy, low back pain, hypothyroidism, bullous pemphigoid, gastroesophageal reflux disease, chronic constipation, C. difficile infection, joint hypermobility syndrome and even hyper Osmia [5,[9][10][11][12]16,[19][20][21].…”

Section: Discussionmentioning

confidence: 99%

“…Irregular vasodilation can lead to an increased shunting of blood through the arterio-venous (AV) anastomosis, causing ischemic damage with release of inflammatory mediators manifested as hyperemia, pain and inflammation of the involved area [2,3]. EM patients have reduced capillary size & density in areas of increased AV anastomosis [11]. Eighty-eight percent of EM patients had dysautonomia with abnormal thermoregulatory skin tests [22].…”

Section: Discussionmentioning

confidence: 99%

“…Physicians use various methods for disease evaluation. Thermoregulatory sweat testing for abnormal thermoregulatory function [22], late blood restoration test (LBRT) to distinguish vasoparalysis in EM from a vasoconstrictive disorder, laser speckled contrast imaging (LSCI) to monitor capillary blood flow [11], capillaroscopy to visualize skin capillary changes [29,30] and thermography to compare acute attacks with asymptomatic periods [6]. Even bone scan documented non-specific findings, opening avenues for radiological testing in future [31].…”

Section: Discussionmentioning

confidence: 99%

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Primary Erythromelalgia Complicated by Cellulitis: A Case Report and Review of Literature

Sharif¹,

Haider²,

Freeman³

et al. 2020

AJMCR

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Background: Erythromelalgia is a rare disease with increasing incidence. It manifests as episodic painful red extremities triggered by heat. External cooling provides temporary symptomatic relief but may lead to complications such as cellulitis. Management includes trigger control, behavioral therapy and pain management. Case Presentation: A 47-year-old African American male presented to the hospital with worsening bilateral lower extremity pain for three months. It was episodic, triggered by running and associated with erythema and swelling. Patient used cold water immersion and air conditioning for pain relief. One week prior to presentation, he developed painful blisters on his feet. On presentation, vital signs were stable, patient was afebrile. Acute infection was ruled out and he was discharged with outpatient rheumatology follow up for erythromelalgia. He returned one week later with worsening symptoms. CT scan of lower extremities indicated bilateral cellulitis. Patient was managed by medicine, dermatology, rheumatology, and podiatry for cellulitis, fungal infection, trench foot and primary erythromelalgia with antibiotics, antifungals, gabapentinoids and behavioral therapy. His infection resolved and pain improved. He was discharged with outpatient rheumatology follow up. Discussion: Erythromelalgia is a highly debilitating disease with episodes of burning erythematous extremities triggered by increase in skin temperature. Patients seek pain relief by excessive external cooling. Pathophysiology involves gain of function mutation in voltage gated sodium channels causing autoregulatory dysfunction of skin. Underlying disease mechanisms are ambiguous and may involve unidentified genetic components and unknown triggers. It is a clinical diagnosis. Therapy requires a multidisciplinary approach. Complications should be promptly addressed given attention next to symptomatic relief. There is a lack of disease specific treatment and complete remission is unlikely. Our patient responded well to gabapentinoids and behavioral therapy.

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Microvascular imaging of primary erythromelalgia

Cited by 2 publications

References 2 publications

Auricular erythermalgia showing excellent response to itraconazole: a case report

Auricular erythermalgia showing excellent response to itraconazole: a case report

Primary Erythromelalgia Complicated by Cellulitis: A Case Report and Review of Literature

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