Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 26, 2019; 7(18): 2808-2814
Published online Sep 26, 2019. doi: 10.12998/wjcc.v7.i18.2808
Pancreatitis, panniculitis, and polyarthritis syndrome caused by pancreatic pseudocyst: A case report
Sungho Jo, Sanghyun Song
Sungho Jo, Sanghyun Song, Department of Surgery, Dankook University Hospital, Dankook University College of Medicine, Cheoan 31116, South Korea
Author contributions: Jo S and Song S performed the surgery, reviewed the literature, and contributed to manuscript drafting.
Informed consent statement: Informed written consent was obtained from the legal guardian (spouse) for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist, and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Sanghyun Song, MD, Assistant Professor, Surgeon, Department of Surgery, Dankook University Hospital, Dankook University College of Medicine, Dongnam-gu Dandae-ro 119, Cheonan-si 31116, Chungcheongnam-do, South Korea. youngmoon77@gmail.com
Telephone: +82-41-5503087 Fax: +82-41-5506034
Received: April 22, 2019
Peer-review started: April 23, 2019
First decision: August 1, 2019
Revised: August 17, 2019
Accepted: August 27, 2019
Article in press: August 26, 2019
Published online: September 26, 2019
Abstract
BACKGROUND

Panniculitis, polyarthritis, and pancreatitis (PPP) syndrome is a triad comprising an extremely rare extra-pancreatic complication of pancreatic disease. Herein, we describe a patient with PPP syndrome caused by fistula formation between the inferior vena cava (IVC) and pancreatic pseudocyst.

CASE SUMMARY

A 64-year-old man visited the hospital with bilateral leg pain that began one week prior. He had no specific diseases, except hypertension. His vital signs were normal. Blood test revealed the following findings: White blood cell count, 28690/µL; amylase level, 9055 U/L; lipase level, 2089 U/L; and C-reactive protein level, 12.94 mg/dL. Computed tomography of the pancreas revealed recent acute pancreatitis. Nonsteroidal anti-inflammatory drugs were administered with no improvement. After steroid administration, pain slightly improved. Skin lesions were diagnosed as panniculitis. Bone scan and knee magnetic resonance imaging revealed osteoarthritis and bone marrow infarctions. Surgical treatment was considered; total pancreatectomy with splenectomy was performed. A pseudocyst was present posterior to the head of the pancreas, forming a fistula with the suprarenal IVC. After surgery, amylase and lipase levels decreased. However, the patient died of an uncontrolled infection on the 13th postoperative day.

CONCLUSION

PPP syndrome should be suspected when accompanied by skin and joint lesions. Delays in diagnosis could have catastrophic consequences.

Keywords: Panniculitis, Polyarthritis, Pancreatitis, Fistula, Case report

Core tip: Panniculitis, polyarthritis, and pancreatitis syndrome must be considered when skin lesions and joint symptoms are associated with suspected pancreatitis. Treatment of the causative pancreatic disease is helpful in relieving symptoms. Surgical treatment is needed when fistulas of the pancreatic duct and systemic circulation formed. Delays in diagnosis could have catastrophic consequences.