Erythromelalgia Presenting with Premature Exfoliation of Primary Teeth: A Diagnostic Dilemma

Erythromelalgia is an extremely rare neurovascular disorder, characterized by symptoms of red, hot, and painful extremities. There is considerable confusion regarding the etiology and pathogenesis of this condition, and the diagnosis is essentially a clinical one. This condition may occur in isolation or in association with other myeloproliferative disorders. Unfortunately, no therapy is effective consistently in managing the symptoms, although early diagnosis can aid in psychological counseling and minimizing the frequency and severity of the attacks. The purpose of this report was to describe the case of a child presenting with premature loss of primary teeth and the difficulties in determining the final diagnosis of erythromelalgia, which responded positively to low-grade aspirin therapy.