Pancreatitis, polyarthritis and panniculitis syndrome

Azar, Lama; Chatterjee, Soumya; Schils, Jean

doi:10.1016/j.jbspin.2013.08.003

Cited by 11 publications

(11 citation statements)

References 2 publications

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“…Much rarer the triad of pancreatitis, subcutaneous tissue inflammation (panniculitis) and polyarthritis, often referred to as “Pancreatitis-Panniculitis-Polyarthritis-Syndrome” or “PPP-syndrome” could be observed. In addition to our case we found 32 well-documented cases in current literature [1] , [5] , [6] , [7] , [8] , [9] , [10] , [11] . Reports are limited to case-reports and speculations about pathogenesis.…”

Section: Literature Review and Discussionsupporting

confidence: 76%

Pancreatitis, panniculitis and polyarthritis (PPP-) syndrome caused by post-pancreatitis pseudocyst with mesenteric fistula. Diagnosis and successful surgical treatment. Case report and review of literature

Dieker

Derer

Schneider

et al. 2017

International Journal of Surgery Case Reports

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Section: Literature Review and Discussionsupporting

confidence: 76%

Pancreatitis, panniculitis and polyarthritis (PPP-) syndrome caused by post-pancreatitis pseudocyst with mesenteric fistula. Diagnosis and successful surgical treatment. Case report and review of literature

Dieker

Derer

Schneider

et al. 2017

International Journal of Surgery Case Reports

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“…In literature 64 cases of PPP syndrome are reported, 34 patients with pancreatitis [2] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , 30 patients with pancreatic cancer [3] ( Table 1 ).…”

Section: Discussionmentioning

confidence: 99%

Pancreatic disease, panniculitis, polyarthrtitis syndrome successfully treated with total pancreatectomy: Case report and literature review

Ferri

Ielpo

Durán

et al. 2016

International Journal of Surgery Case Reports

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“… 113 [ 128 ] 67 m chronic pancreatitis death two months after first skin lesion 114 [ 12 ] 69 m pancreatic ACC resolution after distal pancreatectomy and adjuvant radio-chemotherapy, follow-up 6 months 115 [ 129 ] 29 f acute pancreatitis resolution 116 [ 130 ] 29 m pancreatic pseudocyst-portal vein fistula no new lesions after surgery 117 [ 131 ] 75 m pancreatic tumor Death several months after first skin lesion 118 [ 131 ] 39 m chronic pancreatitis with pseudocyst n.r. 119 [ 132 ] 62 m acute pancreatitis resolution 120 [ 133 ] 77 f pancreatic tumor death five months after first skin lesion 121 [ 134 ] 54 f chronic pancreatitis resolution after ESWL and endoscopic dilation of the pancreatic duct 122 [ 135 ] 47 f acute pancreatitis resolution 123 [ 136 ] 7 m acute pancreatitis n.r. 124 [ 137 ] …”

Section: Case Presentationmentioning

confidence: 99%

Pancreatic panniculitis in a patient with pancreatic-type acinar cell carcinoma of the liver – case report and review of literature

et al. 2016

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BackgroundPancreatic panniculitis is a rare condition, which has only been described in relation with pancreatic diseases up to now. It is characterized by necrotizing subcutaneous inflammation and is thought to be triggered by adipocyte necrosis due to systemic release of pancreatic enzymes with consecutive infiltration of neutrophils. We present the first case of a patient with pancreatic panniculitis caused by pancreatic-type primary acinar cell carcinoma (ACC) of the liver and without underlying pancreatic disease.Case presentationA 73-year old Caucasian female patient was referred to our department with painful cutaneous nodules persisting for eight weeks and with marked lipasemia (~15000 U/l; normal range <60 U/l). Four weeks prior, several liver lesions had been detected. Empiric treatment with steroids did not show any effect. A biopsy of the skin nodules revealed “pancreatic” panniculitis, while abdominal imaging with ultrasound, computed tomography and magnetic resonance imaging detected no abnormal pancreatic findings. Ultrasound-guided biopsy of the liver lesions showed infiltrates of an ACC. The patient died soon thereafter. Autopsy failed to reveal any other primary for the ACC, so that a pancreatic-type ACC of the liver was diagnosed by exclusion.One hundred thirty cases of pancreatic panniculitis published within the last 20 years are reviewed. ACC of the pancreas is the most common underlying neoplastic condition. Patients with associated neoplasm are significantly older, take longer to be diagnosed and have higher lipase levels than patients with underlying pancreatitis.Extrapancreatic pancreatic-type ACC is very rare, but shows the same biological features as ACC of the pancreas. It is believed to develop from metaplastic or ectopic pancreatic tissue. Up to now, no pancreatic panniculitis in extrapancreatic ACC has been described.ConclusionPancreatic panniculitis should always be included in the differential diagnosis of lipolytic panniculitic lesions. It can be regarded as a facultative paraneoplastic phenomenon.When suspected, a thorough work-up for identification of the underlying disease is mandatory and extrapancreatic lesions (e.g. liver) should also be considered. While administration of octreotide or steroids can sometimes alleviate symptoms, immediate treatment of the associated condition is the only effective management option.

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Pancreatitis, polyarthritis and panniculitis syndrome

Cited by 11 publications

References 2 publications

Pancreatitis, panniculitis and polyarthritis (PPP-) syndrome caused by post-pancreatitis pseudocyst with mesenteric fistula. Diagnosis and successful surgical treatment. Case report and review of literature

Pancreatitis, panniculitis and polyarthritis (PPP-) syndrome caused by post-pancreatitis pseudocyst with mesenteric fistula. Diagnosis and successful surgical treatment. Case report and review of literature

Pancreatic disease, panniculitis, polyarthrtitis syndrome successfully treated with total pancreatectomy: Case report and literature review

Pancreatic panniculitis in a patient with pancreatic-type acinar cell carcinoma of the liver – case report and review of literature

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