Editor-in-Chief: Alaa Abd-Elsayed, MD, PhD
BACKGROUND: Pain is a usual symptom in patients with POEMS syndrome. The pain occurs in the superior and inferior limbs and it is featured as numbness, weakness, and tingling that worsens with movement of the affected limb.
CASE REPORT: A 58 year-old-woman began to feel numbness, tingling, and burning in the lower members with progression to her upper extremities, hyperpigmentation, and cutaneous sclerosis. She was in treatment for chronic inflammatory demyelinating polyneuropathy (CIDP) but the symptoms worsened and she was admitted to the hospital. Her laboratory exams showed increases in pro-BNP (N-terminal prohormone of brain natriuretic peptide), platelets, B-2-microglobulin, monoclonal IgG lambda, thyroid stimulating hourmone (TSH), and decreases in T4 and cortisol levels. Computed tomography angiography showed moderate bilateral pleural effusion, pericardial effusion, hepatomegaly, and severe pulmonary hypertension. Thus, she was diagnosed with POEMS syndrome. She reported constant burning, paresthesia, and tingling in both hands with a Visual Analog Scale (VAS) score of 8 of 10, while taking daily doses of 1800 mg of gabapentin, 400 mg of carbamazepine, 4 g of dipyrone, and 100 mg of amantadine. Topical lidocaine 2% + amitriptyline 4% cream was prescribed twice a day. After 2 days of using the cream, the patient reported significant pain improvement, and after 7 days her VAS pain score was 1 of 10. Afterwards, the patient was discharged for ambulatory follow-up.
CONCLUSION: POEMS syndrome is a paraneoplasic disease whose diagnosis can be confused with CIDP at the beginning. The treatment is done with radiotherapy, plasmapheresis, intravenous immunoglobulin, interferon alpha, corticosteroids, azathioprine, and autologous stem cell transplantation. The pain management is complex; therefore, following the usual neuropathic pain treatment guidelines with the addition of topical agents can be a good option.
KEY WORDS: Neuropathic pain, pain, POEMS syndrome