Red Ear Syndrome is a burning sensation and erythema of the ear, associated with a various number of disorders including migraine, trigeminal neuralgia, autoimmune disorders etc. Theories for RES pathophysiology have developed from current understandings of comorbid conditions. Characterizing the underlying mechanism of RES is crucial for defining effective treatments. Three caucasian patients, ages 15, 47, and 67Â years, with migraine, one with erythromelalgia are reported in this manuscript. RES pathophysiology is not fully understood due to its variable clinical presentation and numerous comorbid conditions, making it difficult to identify effective treatments. RES seems to be largely treatment-resistant, and most options involve treating the associated disorders and minimizing pain. Further investigation of future cases should lead to a more comprehensive understanding of the fundamental cause of RES and, hopefully, successful treatments.
Erythromelalgia (EM) is a rare disease characterized by recurrent episodes of erythema, pain, and burning dysesthesia, most often involving the extremities. A rare, atypical presentation involves the face. There are no current guidelines, with overall lack of consensus for the treatment of EM, and therapies are often formulated on a case-by-case basis. We describe the case of a 48-year-old woman who presented with facial EM causing erythema and burning pain with significant impact on quality of life, and was successfully treated with stellate ganglion block. This case highlights that stellate ganglion blocks can be an effective treatment option for patients with facial EM, alongside other conservative therapies that may include medical management.
The evaluation and management of childhood pain syndromes of neuromuscular origin have distinct challenges, as the patterns of disease presentation and the ability of a child to describe symptoms may differ from that of an adult. Advances in scientific and clinical knowledge are leading to significant progress in the care of affected children. The genetic origins of Fabry disease and the inherited form of erythromelalgia are better understood. The increasing interest in neuroimmunology among pediatric neurologists has led to more sophisticated diagnostic and therapeutic approaches. Treatment protocols for complex regional pain syndrome have become more standardized. In addition, investigations continue into potential new interventions for metabolic muscle diseases such as McArdle disease and carnitine palmitoyl transferase deficiency type II. In the years to come, children with pain of neuromuscular origin will have access to more precise diagnostic tools and novel therapies that would alleviate this particularly distressing category of disease.
