OBJECTIVE: To report a case of erythromelalgia in an adolescent patien
t successfully treated with nitroprusside. CASE SUMMARY: A 15-year-old
girl with erythromelalgia resistant to aspirin therapy received an in
fusion of nitroprusside. The response of the erythromelalgia to nitrop
russide was dramatic, With complete pain resolution within 17 hours af
ter the start of therapy. No relapse of erythromelalgia was seen when
nitroprusside was discontinued and the patient remained well after 6 m
onths. DISCUSSION: This case adds to existing literature substantiatin
g the benefit of nitroprusside for the treatment of erythromelalgia in
pediatric patients, Erythromelalgia in children may represent a diffe
rent disease entity than that seen in adults, which is commonly respon
sive to aspirin therapy. The pathogenesis of erythromelalgia is unclea
r and precludes formulating a proposed mechanism by which nitroprussid
e has benefit in children. CONCLUSIONS: Nitroprusside is valuable for
erythromelalgia resistant to aspirin therapy in pediatric patients. Be
cause of unanswered questions regarding the disease, aspirin remains t
he agent of first choice in all patients with this rare disease.