Poems Syndrome With Vasa Nervorum Vasculitis: A Case Report
POEMS syndrome is a rare multisystemic disorder which includes polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. The disease is more frequent in Japan where it is known as Crow-Fukase syndrome. Although pathogenesis remains unclear, there is evidence that vasculopathy may have an important role. We report the case of a 45-year-old woman with a 2 year history of progressive and symmetric distal paresthesias, muscle weakness and atrophy that rendered her severely quadriparetic and bedridden. Electrophysiological study showed a severe axonal and demyelinating polyneuropathy with evidence of active denervation. Additional investigation disclosed IgG-lambda monoclonal serum protein, skin hyperpigmentation, diabetes mellitus, increased cerebrospinal fluid protein, organomegaly (liver and kidney) and multiple osteoclastic lesions involving the second and third lumbar vertebra, the hip, the femur and the tibia. Biopsy of the osteoclastic vertebra revealed a marked lymphoplasmocytic infiltrate. The sural nerve biopsy showed a complete loss of the large myelinated fibres and a moderate reduction of the small ones with signs of Wallerian-like degeneration. Furthermore, epineurial small vessel destructuration and enlargement and a perivascular infiltration of inflammatory cells in one of them was present, compatible with a previous vascular inflammatory disease. In accordance with this picture, immunohistochemical study of glabrous skin double marked for neural and vascular structures showed a marked loss of Meissner corpuscles and myelinated nerve fibers with evident anomalies of blood vessels. Blood examination did not show cryoglobulin, anti-nucleus (ANA) or anti-double strand DNA antibodies. The vascular abnormalities in nerve biopsy or in other tissue biopsies are not frequently described in POEMS syndrome. Our case, as reported by other authors, suggests that microangiopathy could be responsible for peripheral neuropathy and for systemic manifestations of POEMS syndrome.
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Document Type: Abstract
Affiliations: Department of Neurological Sciences, University of Naples “Federico II”
Publication date: March 1, 2001