• Yanqiu Hou, Suyu Jiang, Lu Zhang, Yan Wang, Liubo Zhang, Hongyu Bao, Qingqi Meng, Xue Han, Wanru Chen, Tiantian Li, Jie Peng, Yilin Zhu, Rong Huang, Jingan Liu, Jianning Wang, Chao Fang, and Xiaofeng Shi.
• Second Affiliated Hospital of Nanjing Medical University, Nanjing, China.
• Medicine (Baltimore). 2022 Feb 4; 101 (5): e28651e28651.

RationalePOEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is a rare and complicated disease related to multiple organs and systems. Here, we report a case of systemic mastocytosis (SM) that was misdiagnosed as a POEMS syndrome.Patient ConcernsA 42-year-old man presented with skin changes, diarrhea, and limb numbness.DiagnosesPositron emission tomography/computed tomography revealed extravascular volume overload, organomegaly, lymphadenopathy, and bone lesions with mixed lesions of osteosclerosis and osteolysis. Therefore, POEMS syndrome was suspected. Further histopathological and immunohistochemical examination of the bone marrow, lymph nodes, and gastric mucosa suggested a diagnosis of mastocytosis. The c-Kit D816V mutation confirmed the diagnosis of SM.InterventionsThe patient received the treatment of pegylated interferon-alpha weekly and glucocorticoid daily.OutcomesThe symptoms relieved significantly.LessonsThere are many similar features between POEMS syndrome and SM, probably leading to misdiagnosis. This study analyzed the different points between them which can provide help for differentiation.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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