Vascular acrosyndromes in young adult population. Definition of clinical symptoms and connections to joint hypermobility

Vounotrypidis, Periklis; Pyrpasopoulou, Athina; Sakellariou, G; Zisopoulos, Dimitrios; Kefala, Nikoleta; Oikonomou, Dimitrios; Stefanis, Constantinos; Aslanidis, Spyros; Bermperidis, Charalambos; Pappas, Periklis

doi:10.1007/s10067-019-04627-w

Cited by 2 publications

(1 citation statement)

References 29 publications

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“…"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%

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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