Erythermalgia Involving the Face Alone: Two Case Reports

Liu, Juan-Hua; ZhiSheng, Weng; Luo, Dan; Zhao, Yanyang; Wu, Huihui; Huang, Ying-Biao

doi:10.11607/ofph.2139

Cited by 3 publications

(13 citation statements)

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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%

“…Signs and symptoms of erythromelalgia are intermittent, lasting hours to days based on individual variability. Most commonly involved areas include distal extremities, but involvement of face, ears and cheeks is also seen [2,12,14]. Symmetrical bilateral involvement is more common than asymmetrical unilateral symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…Discovery of mutant sodium channels was a game changer as patients showed improvement with partial sodium channel blockers e.g. mexiletine and lidocaine combined with other modalities of pain treatment [3,14]. Topical formulations like amitriptyline-ketamine, lidocaine, capsaicin, midodrine and neuronal calcium channels modulators like gabapentin have also been used [3,12].…”

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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“…[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%

Section: Discussionmentioning

confidence: 99%

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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“…There are a few rare kinds of diseases, such as erythromelalgia (EM, also called erythermalgia), 1 – 5 red ear syndrome (RES), 6 – 8 and facial flushing, which may present as episodic auricular erythema associated with pain and warmth.…”

Section: Introductionmentioning

confidence: 99%

“… 1 – 5 It always involves the extremities symmetrically, mainly the hands and feet, and may extend to other body parts such as face, neck, ears, nose, tongue, and scrotum. 1 – 5 Sole involvement of EM on vulvae, ears as well as on checks had also been reported respectivelys. 2 , 4 , 5 Its triggering factors include touch, exertion, heat stimuli, movements, stress, raging, and so on.…”

Section: Introductionmentioning

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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Erythromelalgia in a patient with feet erythema and cyanosis

2023

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Erythromelalgia is a rare disease characterised by a triad of a clinical syndrome of redness, warmth and painful extremities. We present the case of a male adolescent with no prior medical history who presents to our family medicine clinic with a 3-month history of bilateral feet erythema followed by episodes of cyanosis in bilateral toes. Given his history, the findings on clinical examination, and the lack of any pathology on the diagnostic testing, the patient is diagnosed with erythromelalgia. He is then counselled on both pharmacological and non-pharmacological treatments for his condition and is discharged on non-pharmacological treatment options such as leg elevation, cooling with a fan and limiting exposure to heat. The patient is also advised to perform an annual complete blood count given the association of erythromelalgia with myeloproliferative disorders.

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Erythermalgia Involving the Face Alone: Two Case Reports

Cited by 3 publications

References 0 publications

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

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

Auricular erythermalgia showing excellent response to itraconazole: a case report

Erythromelalgia in a patient with feet erythema and cyanosis

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