Burning pain attacks and red skin in a young womanA 21-year-old woman presented with a five-year history of burning pain, redness, swelling and heat in her feet. The medical history disclosed palmar hyperhidrosis. Her family history was unremarkable. Examination showed erythema, heat and marked swelling in her feet, which improved after gabapentin and acetylsalicylic acid treatment (Figure). An extensive evaluation for secondary causes was unremarkable.
Examination for erythromelalgia. Note the presence of marked erythema, heat and swelling in feet (A, B) with marked response to acetylsalicylic acid and gabapentin treatment after three weeks (C, D).
Erythromelalgia is characterized by recurrent attacks of intense pain, redness, warmth and swelling, localized to the distal extremities due to secondary etiologies such as myeloproliferative or rheumatologic disorders, drugs (bromocriptine; calcium channel blockers)1, or as primary erythromelalgia2 caused by mutations in the SCN9A gene, coding sodium channel subtype Nav1.7.
References
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1 Tang Z, Chen Z, Tang B, Jiang H. Primary erythromelalgia: a review. Orphanet J Rare Dis. 2015;10:127. https://doi.org/10.1186/s13023-015-0347-1
» https://doi.org/10.1186/s13023-015-0347-1 -
2 McDonnell A, Schulman B, Ali Z, Dib-Hajj SD, Brock F, Cobain S et al. Inherited erythromelalgia due to mutations in SCN9A: natural history, clinical phenotype and somatosensory profile. Brain. 2016;139(4):1052-65. https://doi.org/10.1093/brain/aww007
» https://doi.org/10.1093/brain/aww007
