Journal of Echocardiography

, Volume 16, Issue 4, pp 192–193 | Cite as

Kohlmeier–Degos disease with constrictive pericarditis and atrial fibrillation

  • Stephani C. Wang
  • Hiren Patel
  • Lee S. Shapiro
  • Sulagna Mookherjee
Case image in cardiovascular ultrasound

Cardiovascular ultrasound

Kohlmeier–Degos disease (KD), also known as malignant atrophic papulosis, is a rare thrombo-obliterative vasculopathy with poor prognosis. There are only less than 50 living patients currently around the world [1]. The exact etiology is unclear, but hypotheses include familial inheritance, vasculitis, and aberrant proliferation of vascular endothelium leading to thrombosis which have been implicated. However, cardiac involvement is very uncommon, primarily described as the development of pericardial effusions with possible pericardial thickening [2].

The echocardiographic images shown here are from a 26-year-old man with progressive KD disease leading to the development of constrictive pericarditis. He was diagnosed with the syndrome at age 17; since then, his disease course has been complicated by multi-organ involvement with development of bowel obstruction, restrictive lung disease with recurrent pleural effusions, pulmonary hypertension, and systolic heart...


Compliance with ethical standards

Conflict of interest

Stephani Wang, Hiren Patel, Lee Shapiro, and Sulagna Mookherjee declare that they have no conflict of interest.

Human rights statements and informed consent

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1964 and later revisions. Informed consent was obtained from all patients for being included in the study.


  1. 1.
    Theodoridis A, Makrantonaki E, Zouboulis CC. Malignant atrophic papulosis (Köhlmeier-Degos disease)—a review. Orphanet J Rare Dis. 2013;8:10.CrossRefGoogle Scholar
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    Oliver B, Boehm M, Rosing DR, et al. Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman. J Am Acad Dermatol. 2016;75(6):1274–7.CrossRefGoogle Scholar
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    Shapiro LS, Toledo-Garcia AE, Farrell JF. Effective treatment of malignant atrophic papulosis (Köhlmeier-Degos disease) with treprostinil—early experience. Orphanet J Rare Dis. 2013;8:52.CrossRefGoogle Scholar

Copyright information

© Japanese Society of Echocardiography 2018

Authors and Affiliations

  • Stephani C. Wang
    • 1
  • Hiren Patel
    • 2
  • Lee S. Shapiro
    • 3
  • Sulagna Mookherjee
    • 2
  1. 1.Department of MedicineAlbany Medical CollegeAlbanyUSA
  2. 2.Division of Cardiology, Department of MedicineAlbany Medical CollegeAlbanyUSA
  3. 3.Center for RheumatologySaratoga SpringsUSA

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