POEMS syndrome mimicking diabetic peripheral neuropathy: a case report and literature review
- 1The First Affiliated Hospital of Guangxi Medical University, Nanning, China
- 2The Second People’s Hospital of Nanning, Nanning, China
- 3Guangxi Medical University, Nanning, China
Abstract
POEMS syndrome is a rare multisystem plasma cell disorder characterized by peripheral neuropathy and a broad spectrum of systemic manifestations. Because peripheral neuropathy may coexist with common metabolic disorders, POEMS syndrome can be easily overlooked or misdiagnosed. We report the case of a 64-year-old woman with a 2-year history of diabetes mellitus who presented with progressive numbness of all four limbs for more than 11 months and fatigue for approximately 1 year. She was initially diagnosed with diabetic peripheral neuropathy and treated with glucose-lowering and neuroprotective therapy, but her neurological symptoms persisted and progressed. Further evaluation demonstrated demyelinating peripheral neuropathy with axonal involvement, an IgA-λ monoclonal protein, a clonal plasma cell population in the bone marrow, markedly elevated plasma vascular endothelial growth factor (VEGF), bilateral papilledema, lymphadenopathy, lower-extremity edema, and characteristic skin changes. Based on the combination of mandatory, major, and minor diagnostic features, POEMS syndrome was diagnosed. The patient initially received lenalidomide plus dexamethasone (RD), but lenalidomide was discontinued after the development of a generalized inflammatory erythematous rash with scaling and pruritus during the second treatment cycle. Treatment was subsequently changed to daratumumab, bortezomib, and dexamethasone (Dara + VD). During Dara + VD treatment, serum M-protein became undetectable, plasma VEGF decreased from 842.29 pg/mL before treatment to 836 pg/mL after the first cycle, and edema, fatigue, abdominal distension, and peripheral neuropathic symptoms improved. However, the patient was discharged after receiving only the first administration of the fourth treatment cycle. Three days after discharge, she developed generalized seizures with loss of consciousness and subsequently experienced severe systemic complications, including pneumonia, sepsis, septic shock, renal failure, and heart failure. She eventually died of multiple organ dysfunction syndrome. This case highlights the diagnostic challenge of POEMS syndrome in patients with pre-existing diabetes mellitus. Progressive or atypical peripheral neuropathy that is disproportionate to the presumed metabolic disorder should prompt evaluation for monoclonal plasma cell disorders and POEMS syndrome, particularly when accompanied by edema, skin changes, lymphadenopathy, papilledema, or elevated VEGF. In this patient, Dara + VD was associated with a short-term hematological and clinical response after lenalidomide intolerance; however, the subsequent fatal course precludes conclusions regarding its long-term efficacy.
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BibTeX
@article{chen2026poems,
title = {{POEMS syndrome mimicking diabetic peripheral neuropathy: a case report and literature review}},
author = {Yemei Chen and Chuxuan Fang and Shanfei Liang and Xinfei Xie and Shuangyi Tang and Zongxuan Huang},
journal = {Frontiers in Medicine},
year = {2026},
month = oct,
doi = {10.3389/fmed.2026.1993357},
url = {https://doi.org/10.3389/fmed.2026.1993357},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - POEMS syndrome mimicking diabetic peripheral neuropathy: a case report and literature review
AU - Yemei Chen
AU - Chuxuan Fang
AU - Shanfei Liang
AU - Xinfei Xie
AU - Shuangyi Tang
AU - Zongxuan Huang
PY - 2026
DA - 2026/10/21
JO - Frontiers in Medicine
DO - 10.3389/fmed.2026.1993357
UR - https://doi.org/10.3389/fmed.2026.1993357
AB - POEMS syndrome is a rare multisystem plasma cell disorder characterized by peripheral neuropathy and a broad spectrum of systemic manifestations. Because peripheral neuropathy may coexist with common metabolic disorders, POEMS syndrome can be easily overlooked or misdiagnosed. We report the case of a 64-year-old woman with a 2-year history of diabetes mellitus who presented with progressive numbness of all four limbs for more than 11 months and fatigue for approximately 1 year. She was initially diagnosed with diabetic peripheral neuropathy and treated with glucose-lowering and neuroprotective therapy, but her neurological symptoms persisted and progressed. Further evaluation demonstrated demyelinating peripheral neuropathy with axonal involvement, an IgA-λ monoclonal protein, a clonal plasma cell population in the bone marrow, markedly elevated plasma vascular endothelial growth factor (VEGF), bilateral papilledema, lymphadenopathy, lower-extremity edema, and characteristic skin changes. Based on the combination of mandatory, major, and minor diagnostic features, POEMS syndrome was diagnosed. The patient initially received lenalidomide plus dexamethasone (RD), but lenalidomide was discontinued after the development of a generalized inflammatory erythematous rash with scaling and pruritus during the second treatment cycle. Treatment was subsequently changed to daratumumab, bortezomib, and dexamethasone (Dara + VD). During Dara + VD treatment, serum M-protein became undetectable, plasma VEGF decreased from 842.29 pg/mL before treatment to 836 pg/mL after the first cycle, and edema, fatigue, abdominal distension, and peripheral neuropathic symptoms improved. However, the patient was discharged after receiving only the first administration of the fourth treatment cycle. Three days after discharge, she developed generalized seizures with loss of consciousness and subsequently experienced severe systemic complications, including pneumonia, sepsis, septic shock, renal failure, and heart failure. She eventually died of multiple organ dysfunction syndrome. This case highlights the diagnostic challenge of POEMS syndrome in patients with pre-existing diabetes mellitus. Progressive or atypical peripheral neuropathy that is disproportionate to the presumed metabolic disorder should prompt evaluation for monoclonal plasma cell disorders and POEMS syndrome, particularly when accompanied by edema, skin changes, lymphadenopathy, papilledema, or elevated VEGF. In this patient, Dara + VD was associated with a short-term hematological and clinical response after lenalidomide intolerance; however, the subsequent fatal course precludes conclusions regarding its long-term efficacy.
SN - 2296-858X
ER -