Pediatric red ear syndrome: A case report of an erythromelalgia type and review of the literature

Cinats, Allison K.; Haber, Richard M.

doi:10.1111/pde.13911

Cited by 11 publications

(15 citation statements)

References 22 publications

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“…She refused these injections and biological agents were not used. The JAK inhibitors, tofacitinib and baricitinib have shown broad prospects in the treatment of psoriasis, psoriatic arthritis, atopic dermatitis, and alopecia areata ( 20 ). Therefore, we decided to use JAK inhibitors to treat this patient.…”

Section: Discussionmentioning

confidence: 99%

Tofacitinib Treatment of Refractory Cutaneous Leukocytoclastic Vasculitis: A Case Report

Zhu

Yang

2021

Front. Immunol.

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IntroductionTo date, there is no treatment with proven efficacy for cutaneous leukocytoclastic vasculitis (CLV). Several reports have suggested that CLV responds favorably to corticosteroids, colchicine, nonsteroidal anti-inflammatory drugs (NSAIDs), azathioprine, and hydroxychloroquine (HCQ). To the best of our knowledge, the oral small molecule Janus kinase inhibitor, tofacitinib, plays an important role in the treatment of autoimmune and inflammatory diseases. Therefore, tofacitinib may be a prospective therapy in patients with CLV.Case PresentationA 29-year-old woman presented to our hospital with a 5-year history of symmetric skin lesions mainly affecting both lower extremities. The results for anti-neutrophil cytoplasmic antibodies (ANCA), anti-extracted nuclear antigens (ENA) autoantibodies, anti-double-stranded deoxyribonucleic acid (dsDNA) antibodies, and antinuclear antibodies (ANA) were all negative. The definite diagnosis of CLV was determined by a skin biopsy. However, the patient exhibited a poor response to prednisone, HCQ, methotrexate, colchicine, azathioprine, and tripterygium wilfordii polyglycoside tablets (TGTs) treatments. She was then treated with oral tofacitinib (5 mg twice daily) and oral prednisone (25 mg daily).OutcomesHer skin lesions gradually improved over a period of 4 weeks. Two months later, the skin ulcers completely resolved. No evidence of recurrence of skin ulcers was observed during a 6-month follow-up.ConclusionWe present the first case of a female patient receiving short-term tofacitinib therapy for refractory CLV. Tofacitinib may be a promising oral alternative for patients with CLV. However, its efficacy and safety require further appraisal through clinical trials.

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Section: Discussionmentioning

confidence: 99%

Tofacitinib Treatment of Refractory Cutaneous Leukocytoclastic Vasculitis: A Case Report

Zhu

Yang

2021

Front. Immunol.

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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%

“…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%

“…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]. Decreased pain perception through targeted Nav1.7 channel antagonism is still experimental whereas mutant potassium channels antagonism and NAD synthesizing mutant NMNAT2 enzyme potentiation is yet unexplored [13,32,35].…”

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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Dermatoses of the External Ear

Kennedy,

Sharma

2024

Rook's Textbook of Dermatology

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The external ear comprises the pinna (auricle), the external auditory canal and the outer surface of the tympanic membrane. Numerous disorders can affect the ear, from childhood to old age. Ear malformations can arise as developmental abnormalities. Traumatic conditions include contusion, haematoma, split earlobes, pseudocyst, keloid and chondrodermatitis. Infections, including viral, bacterial and fungal, may manifest as acute or chronic otitis externa. Ear signs can be a manifestation of an underlying systemic disorder, such as autoimmune and inflammatory disease. Common cancers affecting the ear include basal cell carcinoma and squamous cell carcinoma, the latter carrying a risk of direct and metastatic spread.

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Pediatric red ear syndrome: A case report of an erythromelalgia type and review of the literature

Cited by 11 publications

References 22 publications

Tofacitinib Treatment of Refractory Cutaneous Leukocytoclastic Vasculitis: A Case Report

Tofacitinib Treatment of Refractory Cutaneous Leukocytoclastic Vasculitis: A Case Report

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

Dermatoses of the External Ear

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