Raynaud’s phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report
Abstract Background POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud’s phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not b...
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| Format: | Article |
| Language: | English |
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BMC
2022-05-01
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| Series: | BMC Rheumatology |
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| Online Access: | https://doi.org/10.1186/s41927-022-00258-y |
| _version_ | 1828807425343356928 |
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| author | Fabio Torres-Saavedra Lina León-Sierra |
| author_facet | Fabio Torres-Saavedra Lina León-Sierra |
| author_sort | Fabio Torres-Saavedra |
| collection | DOAJ |
| description | Abstract Background POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud’s phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not been described positive antinuclear antibodies (ANA) and this could lead to a misdiagnosis of autoimmune disease, mainly systemic sclerosis. Case presentation A 47-year-old man presented with color changes on fingertips consistent with biphasic Raynaud’s phenomenon; an antinuclear antibody test was positive (at 1:320 titers in a speckled pattern) with normal nailfold capillaroscopy. Clinical features of systemic sclerosis were absent. Twenty-four months later, the patient presented symmetric sensorimotor demyelinating polyneuropathy, and he was diagnosed with Guillain–Barre syndrome; treatment with intravenous gammaglobulin had an incomplete response. Raynaud’s phenomenon persisted associated with acrocyanosis, white nails, and positive ANA (1:1280 in a nucleolar pattern). POEMS syndrome was suspected, and serum protein electrophoresis (SPEP) was done. The SPEP revealed polyclonal gammopathy, and serum immunofixation showed monoclonal (M)-protein (IgG lambda). Serum vascular endothelial growth factor concentration showed increased levels. The patient was diagnosed with POEMS syndrome, and treatment with lenalidomide and dexamethasone improved the Raynaud’s phenomenon, acrocyanosis, and white nails, but the neurological response was partial. Conclusions POEMS syndrome may mimic clinical manifestations of systemic sclerosis v.g. Raynaud’s phenomenon, skin thickening, telangiectasia, and positive ANA. Raynaud’s phenomenon may precede other clinical manifestations of POEMS syndrome by several months. It is necessary to have a high index of suspicion for the diagnosis, especially in patients with peripheral polyneuropathy and monoclonal paraprotein. The significance of positive ANA in this condition is unknown and deserves further investigation. |
| first_indexed | 2024-12-12T08:24:35Z |
| format | Article |
| id | doaj.art-21a9d1da3bf8465f985787a195e2451d |
| institution | Directory Open Access Journal |
| issn | 2520-1026 |
| language | English |
| last_indexed | 2024-12-12T08:24:35Z |
| publishDate | 2022-05-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Rheumatology |
| spelling | doaj.art-21a9d1da3bf8465f985787a195e2451d2022-12-22T00:31:17ZengBMCBMC Rheumatology2520-10262022-05-01611510.1186/s41927-022-00258-yRaynaud’s phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case reportFabio Torres-Saavedra0Lina León-Sierra1Division of Rheumatology, GRUA Investigation Group, Universidad de AntioquiaInternal Medicine, Universidad Nacional de ColombiaAbstract Background POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud’s phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not been described positive antinuclear antibodies (ANA) and this could lead to a misdiagnosis of autoimmune disease, mainly systemic sclerosis. Case presentation A 47-year-old man presented with color changes on fingertips consistent with biphasic Raynaud’s phenomenon; an antinuclear antibody test was positive (at 1:320 titers in a speckled pattern) with normal nailfold capillaroscopy. Clinical features of systemic sclerosis were absent. Twenty-four months later, the patient presented symmetric sensorimotor demyelinating polyneuropathy, and he was diagnosed with Guillain–Barre syndrome; treatment with intravenous gammaglobulin had an incomplete response. Raynaud’s phenomenon persisted associated with acrocyanosis, white nails, and positive ANA (1:1280 in a nucleolar pattern). POEMS syndrome was suspected, and serum protein electrophoresis (SPEP) was done. The SPEP revealed polyclonal gammopathy, and serum immunofixation showed monoclonal (M)-protein (IgG lambda). Serum vascular endothelial growth factor concentration showed increased levels. The patient was diagnosed with POEMS syndrome, and treatment with lenalidomide and dexamethasone improved the Raynaud’s phenomenon, acrocyanosis, and white nails, but the neurological response was partial. Conclusions POEMS syndrome may mimic clinical manifestations of systemic sclerosis v.g. Raynaud’s phenomenon, skin thickening, telangiectasia, and positive ANA. Raynaud’s phenomenon may precede other clinical manifestations of POEMS syndrome by several months. It is necessary to have a high index of suspicion for the diagnosis, especially in patients with peripheral polyneuropathy and monoclonal paraprotein. The significance of positive ANA in this condition is unknown and deserves further investigation.https://doi.org/10.1186/s41927-022-00258-yRaynaud diseasePOEMS syndromeParaneoplastic polyneuropathyAutoimmune diseaseCase report |
| spellingShingle | Fabio Torres-Saavedra Lina León-Sierra Raynaud’s phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report BMC Rheumatology Raynaud disease POEMS syndrome Paraneoplastic polyneuropathy Autoimmune disease Case report |
| title | Raynaud’s phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report |
| title_full | Raynaud’s phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report |
| title_fullStr | Raynaud’s phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report |
| title_full_unstemmed | Raynaud’s phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report |
| title_short | Raynaud’s phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report |
| title_sort | raynaud s phenomenon and positive antinuclear antibodies as first manifestation of poems syndrome polyneuropathy organomegaly endocrinopathy monoclonal gammopathy and skin changes a case report |
| topic | Raynaud disease POEMS syndrome Paraneoplastic polyneuropathy Autoimmune disease Case report |
| url | https://doi.org/10.1186/s41927-022-00258-y |
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