Hypertensive Emergency, Matlike Telangiectasias, and Calciphylaxis in POEMS Syndrome | Allergy and Clinical Immunology | JAMA Dermatology | JAMA Network
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Observation
June 2014

Hypertensive Emergency, Matlike Telangiectasias, and Calciphylaxis in POEMS Syndrome

Author Affiliations
  • 1Department of Dermatology, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, Ohio
  • 2Department of Medicine, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, Ohio
JAMA Dermatol. 2014;150(6):667-669. doi:10.1001/jamadermatol.2013.7256

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a paraneoplastic syndrome caused by plasma cell dyscrasias. We report hypertensive emergency and acral matlike telangiectasias as novel manifestations of POEMS syndrome (POEMS). Also, to our knowledge, this is the first reported case of calciphylaxis and POEMS occurring in the absence of sclerotic bone lesions or a peripheral monoclonal gammopathy, highlighting the diagnostic challenges posed by this disease.

A woman in her 30s with a 2-year history of distal polyneuropathy and matlike telangiectasias presented with a hypertensive emergency and peak blood pressure of 247/154 mm Hg. Workup revealed a serous pericardial effusion, hepatosplenomegaly, ascites, shotty lymphadenopathy, and diffuse subcutaneous edema; the remainder of her workup findings were negative, including those for rheumatoid factor, antineutrophil cytoplasmic antibodies, antinuclear antibodies, extractable nuclear antigen antibodies panel, serum and urine protein electrophoresis with immunofixation, and brain magnetic resonance imaging. After blood pressure was brought under control, prednisone treatment was empirically initiated, and the ascites improved.

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