Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome
Calciphylaxis is a vascular calcification-cutaneous necrosis syndrome, usually seen in patients with end-stage renal disease and secondary hyperparathyroidism. We report a 57-year-old polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome patient compl...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Karger Publishers
2009-12-01
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| Series: | Case Reports in Neurology |
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| Online Access: | http://www.karger.com/Article/FullText/259906 |
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| author | Akiyo Hineno Tomomi Kinoshita Michiaki Kinoshita Fuyuko Arakura Ko-suke Naito Yasuhiro Shimojima Masayuki Matsuda Kunihiro Yoshida Shu-ichi Ikeda |
| author_facet | Akiyo Hineno Tomomi Kinoshita Michiaki Kinoshita Fuyuko Arakura Ko-suke Naito Yasuhiro Shimojima Masayuki Matsuda Kunihiro Yoshida Shu-ichi Ikeda |
| author_sort | Akiyo Hineno |
| collection | DOAJ |
| description | Calciphylaxis is a vascular calcification-cutaneous necrosis syndrome, usually seen in patients with end-stage renal disease and secondary hyperparathyroidism. We report a 57-year-old polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome patient complicated with extensive skin ulcers due to calciphylaxis. He first noted a painful cutaneous ulcer on his left thigh, and then skin lesions rapidly worsened, resulting in multiple intractable ulcers with gangrene on his legs and trunk in a few months. Serum vascular endothelial growth factor (VEGF) was markedly elevated. Biopsy samples from his skin ulcers showed the deposition of calcium in the medial layer of cutaneous vessels, this finding being compatible with calciphylaxis. This is the second reported case with POEMS syndrome complicated with calciphylaxis. Both patients had no evidence of renal failure, hyperparathyroidism, or clotting disorders. The pathogenic link between POEMS syndrome and calciphylaxis is still unclear, but VEGF is known to regulate vascular calcification, in cooperation with bone morphogenetic proteins. Further, corticosteroid and several proinflammatory cytokines activate nuclear factor-κB pathway, known as the final common pathway leading to vascular calcification. Taken together, we consider that POEMS syndrome can be an independent risk condition for calciphylaxis. |
| first_indexed | 2024-12-22T12:26:45Z |
| format | Article |
| id | doaj.art-8018c07ec1ae42b0b959d4c5c3599c68 |
| institution | Directory Open Access Journal |
| issn | 1662-680X |
| language | English |
| last_indexed | 2024-12-22T12:26:45Z |
| publishDate | 2009-12-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Neurology |
| spelling | doaj.art-8018c07ec1ae42b0b959d4c5c3599c682022-12-21T18:25:47ZengKarger PublishersCase Reports in Neurology1662-680X2009-12-0111475310.1159/000259906259906Calciphylaxis as a Catastrophic Complication in a Patient with POEMS SyndromeAkiyo HinenoTomomi KinoshitaMichiaki KinoshitaFuyuko ArakuraKo-suke NaitoYasuhiro ShimojimaMasayuki MatsudaKunihiro YoshidaShu-ichi IkedaCalciphylaxis is a vascular calcification-cutaneous necrosis syndrome, usually seen in patients with end-stage renal disease and secondary hyperparathyroidism. We report a 57-year-old polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome patient complicated with extensive skin ulcers due to calciphylaxis. He first noted a painful cutaneous ulcer on his left thigh, and then skin lesions rapidly worsened, resulting in multiple intractable ulcers with gangrene on his legs and trunk in a few months. Serum vascular endothelial growth factor (VEGF) was markedly elevated. Biopsy samples from his skin ulcers showed the deposition of calcium in the medial layer of cutaneous vessels, this finding being compatible with calciphylaxis. This is the second reported case with POEMS syndrome complicated with calciphylaxis. Both patients had no evidence of renal failure, hyperparathyroidism, or clotting disorders. The pathogenic link between POEMS syndrome and calciphylaxis is still unclear, but VEGF is known to regulate vascular calcification, in cooperation with bone morphogenetic proteins. Further, corticosteroid and several proinflammatory cytokines activate nuclear factor-κB pathway, known as the final common pathway leading to vascular calcification. Taken together, we consider that POEMS syndrome can be an independent risk condition for calciphylaxis.http://www.karger.com/Article/FullText/259906CalciphylaxisVascular endothelial growth factor (VEGF)POEMS syndromeVascular calcification |
| spellingShingle | Akiyo Hineno Tomomi Kinoshita Michiaki Kinoshita Fuyuko Arakura Ko-suke Naito Yasuhiro Shimojima Masayuki Matsuda Kunihiro Yoshida Shu-ichi Ikeda Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome Case Reports in Neurology Calciphylaxis Vascular endothelial growth factor (VEGF) POEMS syndrome Vascular calcification |
| title | Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome |
| title_full | Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome |
| title_fullStr | Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome |
| title_full_unstemmed | Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome |
| title_short | Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome |
| title_sort | calciphylaxis as a catastrophic complication in a patient with poems syndrome |
| topic | Calciphylaxis Vascular endothelial growth factor (VEGF) POEMS syndrome Vascular calcification |
| url | http://www.karger.com/Article/FullText/259906 |
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