Between Episodes of Erythromelalgia: A Spectrum of Colors

Davis, Mark D.P.; Wilkins, Francesca; Rooke, T. W.

doi:10.1001/archderm.142.8.1085

Cited by 13 publications

(7 citation statements)

References 5 publications

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“…The clinical presentation was consistent with that described in adult patients: during symptomatic periods, the affected areas were red, hot, and painful 3 ; between these episodes, they were frequently cool, cyanotic, and mildly uncomfortable. 15 Underlying disorders previously described in association with erythromelalgia 2,3,5,16,17 were not present in this group of pediatric patients; in particular, none had myeloproliferative disease. Only one patient had hypertension.…”

Section: Discussionmentioning

confidence: 61%

Pediatric erythromelalgia: A?retrospective review of 32 cases evaluated at Mayo Clinic over a 37-year period

Cook-Norris

Tollefson

Cruz-Inigo

et al. 2012

Journal of the American Academy of Dermatology

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Section: Discussionmentioning

confidence: 61%

Pediatric erythromelalgia: A?retrospective review of 32 cases evaluated at Mayo Clinic over a 37-year period

Cook-Norris

Tollefson

Cruz-Inigo

et al. 2012

Journal of the American Academy of Dermatology

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“…report the following comorbidity percentages as follows: smoking (50%) hypertension (13.7%), hyperlipidemia (11.3%) and diabetes mellitus (2.4%). 2 , 26 , 27 , 29 , 35 - 37 , 65 , 67…”

Section: Frequent Comorbiditiesmentioning

confidence: 99%

Erythromelalgia: a cutaneous manifestation of neuropathy?

Leroux¹

2018

An. Bras. Dermatol.

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The low prevalence of erythromelalgia, classified as an orphan disease, poses diagnostic and therapeutic difficulties. The aim of this review is to be an update of the specialized bibliography. Erythromelalgia is an infrequent episodic acrosyndrome affecting mainly both lower limbs symmetrically with the classic triad of erythema, warmth and burning pain. Primary erythromelalgia is an autosomal dominant inherited disorder, while secondary is associated with myeloproliferative diseases, among others. In its etiopathogenesis, there are neural and vascular abnormalities that can be combined. The diagnosis is based on exhaustive clinical history and physical examination. Complications are due to changes in the skin barrier function, ischemia and compromise of cutaneous nerves. Because of the complexity of its pathogenesis, erythromelalgia should always be included in the differential diagnosis of conditions that cause chronic pain and/or peripheral edema. The prevention of crisis is based on a strict control of triggers and promotion of preventive measures. Since there is no specific and effective treatment, control should focus on the underlying disease. However, there are numerous topical and systemic therapies that patients can benefit from.

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“…1,3,4 Due to small vessel dysregulation, erythromelalgia in some cases may present as a violaceous lesion cool to the touch, similar to Raynaud phenomenon. 8 Furthermore, attempted therapeutic cooling measures may trigger local vasospasm and acrocyanosis. 9 Despite these similarities, there is a distinct pathologic process in each condition: whereas Raynaud phenomenon is due to disordered arteriolar vasoconstriction and triggered mainly by cold exposure, erythromelalgia is a manifestation of unregulated hyperemia that is primarily heat exposureinduced.…”

Section: Discussionmentioning

confidence: 99%

“…9 Despite these similarities, there is a distinct pathologic process in each condition: whereas Raynaud phenomenon is due to disordered arteriolar vasoconstriction and triggered mainly by cold exposure, erythromelalgia is a manifestation of unregulated hyperemia that is primarily heat exposureinduced. 8 Erythromelalgia poses not only a diagnostic but therapeutic challenge with no known cure and often results in a significant impact on quality of life. [1][2][3][4][5] If a presumptive diagnosis can be made in the ED setting, the first steps are to identify and avoid disease triggers such as excessive heat exposure.…”

Section: Discussionmentioning

confidence: 99%

Not Another Presentation of Cellulitis: A Case Report of Erythromelalgia

Winters¹

2022

JETEM

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Cellulitis is among the more common conditions diagnosed and treated within pediatric emergency departments. However, unusual distribution, recurrence, and/or poor response to antibiotics may point towards another pathologic process. This case presentation illustrates a teenager with recurrent episodes of painful erythematous and swollen extremities misdiagnosed repeatedly as cellulitis. Careful history, exam, laboratory evaluation and rheumatologist consultation aided in making the correct diagnosis of erythromelalgia, which was successfully treated with supportive measures. This report reviews a rare presentation of erythromelalgia, as well as its pathophysiology, recommended therapies, and common misdiagnoses.

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Between Episodes of Erythromelalgia: A Spectrum of Colors

Cited by 13 publications

References 5 publications

Pediatric erythromelalgia: A?retrospective review of 32 cases evaluated at Mayo Clinic over a 37-year period

Pediatric erythromelalgia: A?retrospective review of 32 cases evaluated at Mayo Clinic over a 37-year period

Erythromelalgia: a cutaneous manifestation of neuropathy?

Not Another Presentation of Cellulitis: A Case Report of Erythromelalgia

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