Citation

Cook-Norris RH, Tollefson MM, Cruz-Inigo AE, Sandroni P, Davis MDP, Davis DMR. J. Am. Acad. Dermatol. 2012; 66(3): 416-423.

Copyright

(Copyright © 2012, Elsevier Publishing)

DOI

10.1016/j.jaad.2011.01.010

PMID

unavailable

Abstract

BACKGROUND: Erythromelalgia has not been well characterized in the pediatric population.

OBJECTIVE: We sought to review our experience of erythromelalgia in the pediatric age group.

METHODS: We conducted a retrospective review of patients 18 years of age and younger with a diagnosis of erythromelalgia who were examined at Mayo Clinic in Rochester, MN, from 1970 to 2007.

RESULTS: The records of 32 patients (girls, 22 [69%]) were evaluated. Mean age was 14.1 years (range, 5-18 years) and mean time to diagnosis was 5.2 years. Seven patients (22%) had a first-degree relative with erythromelalgia; 4 were from the same family. Physical activity was limited because of discomfort in 21 patients (66%) and school attendance was affected in 11 patients (34%). Noninvasive vascular studies, which compared temperature, laser Doppler flow, and transcutaneous oximetry in the toes, identified vascular abnormalities in 13 (93%) of 14 patients. Neurophysiologic studies with autonomic reflex screening (including quantitative sudomotor axon reflex test and thermoregulatory sweat testing) showed evidence of a small-fiber neuropathy involving the skin in 10 (59%) of 17 patients studied; there was no evidence of large-fiber neuropathy in 20 patients in whom electromyographic and nerve conduction studies were performed. Topical lidocaine was the most commonly prescribed treatment (44%). Fifteen patients were monitored for an average of 9.1 years (median, 5.0 years; range, 0.4-23.7 years). At last follow-up, 5 patients had stable disease, 4 showed improvement, two had resolution, one reported worsening of symptoms, and 3 had died (one suicide). Limitations: Conclusions are limited because this was a retrospective chart review.

CONCLUSION: Erythromelalgia in pediatric patients is associated with substantial morbidity and even death. The majority of cases are not inherited. Most patients studied have associated small-fiber neuropathy. The disease course is variable. A reliable and safe treatment has not been determined. © 2011 by the American Academy of Dermatology, Inc.

Language: en

Keywords

human; physical activity; review; retrospective study; amitriptyline; nortriptyline; venlafaxine; patient monitoring; priority journal; acetylsalicylic acid; paracetamol; follow up; carbamazepine; sweating; diphenhydramine; naproxen; corticosteroid; lidocaine; unindexed drug; gabapentin; cimetidine; phenytoin; propranolol; morphine sulfate; ibuprofen; atenolol; fentanyl citrate; cyproheptadine; cellulitis; capsaicin; fluphenazine; cetirizine; neuropathy; neurologic examination; diltiazem; codeine phosphate; temperature; sympathectomy; nitroprusside sodium; electromyography; indometacin; neurophysiology; nadolol; nifedipine; corticosteroid therapy; nerve conduction; transcutaneous nerve stimulation; oximetry; Raynaud phenomenon; acrocyanosis; doxazosin mesylate; erythermalgia; erythromelalgia; laser Doppler flowmetry; pediatric psychology; small-fiber neuropathy; vascular anomalies