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August 26, 2020

TEMPI Syndrome With Progressive Telangiectasias Associated With Pulmonary Deterioration

Author Affiliations
  • 1David Geffen School of Medicine, Division of Dermatology, Department of Medicine, University of California, Los Angeles, Los Angeles
  • 2Department of Internal Medicine, Kaiser Permanente Los Angeles Medical Center, Los Angeles
JAMA Dermatol. 2020;156(12):1379-1380. doi:10.1001/jamadermatol.2020.2668

TEMPI (telangiectasias, erythrocytosis with elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, intrapulmonary shunting) syndrome is a recently described rare plasma cell neoplasm with paraneoplastic features.1,2 In this case report, we describe a patient with TEMPI syndrome with classic and atypical cutaneous findings that track with the progression of pulmonary symptoms.

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