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June 2018

Improvement in Ulcerative Necrobiosis Lipoidica After Janus Kinase–Inhibitor Therapy for Polycythemia Vera

Author Affiliations
  • 1Department of Dermatology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
JAMA Dermatol. 2018;154(6):733-734. doi:10.1001/jamadermatol.2018.0756

Necrobiosis lipoidica (NL) is a noninfectious, granulomatous, inflammatory skin disorder of uncertain etiology that has been classically associated with diabetes mellitus but often occurs without coexisting abnormalities in glucose metabolism.1 Microangiopathy, collagen devitalization, metabolic alterations, trauma, and aberrant immunity are proposed pathogenic mechanisms thought to cause this disease.2 Polycythemia vera (PCV) is a myeloproliferative disorder in which most patients harbor activating mutations in the Janus kinase (JAK) 2 (JAK2) gene.3 To our knowledge, NL and PCV have not been reported in association. Herein, we describe a patient with generalized ulcerative NL who experienced successful treatment of the skin disease following JAK-inhibitor therapy for concomitant PCV.

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