Successful ultrasound guided stellate ganglion block treatment in a patient with upper limb erythromelalgia
Venue: Dermatologic therapy
Year: 2020

Dear Editor, Erythromelalgia is a rare disease of unknown etiology, characterized by symmetric redness, burning pain, and elevated skin temperature, and its treatment is a challenge. The pain is notably aggravated by exercise, heat, or standing for a long time and alleviated by cold water, decreased temperature, or lying flat. The disease can start at any age, but it is common in young adults. Relief with ice cold water immersion is common, which is the most characteristic sign. In addition, erythromelalgia may be related to genetics, especially the SCN9A gene. The condition is divided into primary and secondary erythromelalgia, and usually occurs in the lower extremities, most commonly the feet, followed by the hands and less commonly in the arms, ears, and face. We report the case of a woman with upper limb erythromelalgia who was treated successfully with ultrasound guided stellate ganglion block (SGB) (operation methods are described in Appendix S1) A 37 year old female patient had a 10 year history of foot and palm pain with heat and exercise; she had sagging limbs and developed swelling and burning pain in the soles of both feet and palms that was accompanied by flushing of the skin and increased local skin temperature. Her pain was relieved when the lower limbs and hands were placed in cold water or in a cool environment. Before admission, the patient took oral gabapentin (300 mg three times a day) and the pain was effectively controlled. However, the pain worsened in the last month before admission, and the visual analog scale was 6 to 8. Physical examination revealed flushing of the skin on the soles of the feet, palms, and fingers; elevated temperature; decreased blood pressure; mild swelling of the feet; and no pitting edema. Laboratory tests showed a normal red blood cell count, platelet count, white blood cell count, and blood glucose. Raynaud's disease, thromboangiitis obliterans, neuropathy, tumor, and connective tissue disease were further ruled out, and primary erythromelalgia was diagnosed (the diagnostic procedure is described in Appendix S2) This patient underwent SGB, alternating left and right, twice a day. After a total of 10 ultrasound guided SGB treatments, the pain in both hands was completely relieved. To treat the erythromelalgia of the lower extremities, a lumbar 4 sympathetic block was performed, and the postoperative pain was partially relieved. The pain was still not completely relieved until discharge. Erythromelalgia is a rare disease of unknown etiology, characterized by symmetric redness, burning pain, and elevated skin temperature. Several treatments have been reported, including drug treatments such as aspirin, pregabalin, lidocaine, selective serotonin reuptake inhibitors, and gabapentin, and surgical treatments including lumbar sympathetic block, epidurals, and sympathectomy, with inconsistent outcomes across the literature. SGB is the oldest and most commonly used treatment for upper limb pain and headache, and some studies have reported that SGB also has the functions of regulating endocrine and autonomic nervous dysfunction, vasoconstriction, and relaxation. In addition, SGB under ultrasound guidance has been widely used in clinical practice due to its accurate localization, simple operation, low price, and minimal complications. Therefore, SGB may be a potentially effective treatment for erythromelalgia of the upper extremities.