title: Pancreatic disease, panniculitis, polyarthrtitis syndrome successfully treated with total pancreatectomy: Case report and literature review.
abstract: BACKGROUND
  Pancreatic disease can be complicated by extrabdominal manifestations such as panniculitis and polyarthritis. The symptomatic triad comprising pancreatic disease, panniculitis and polyarthritis is also known as PPP syndrome and is characterized by severe chronic sequels and high mortality rate. We describe a case of PPP syndrome successfully treated with spleen preserving total pancreatectomy; in addition we performed a literature review.
  
  
  METHODS
  A 67 years old male presented panniculitis and polyarthritis without clinical abdominal symptoms. Clinical presentation, laboratory values and radiological findings demonstrated an acute pancreatitis and a pancreatic cancer was suspected; failure of conservatory treatments and high suspicious of malignancy led to perform a spleen preserving total pancreatectomy. Finally histological examination excluded a pancreatic cancer and confirmed a chronic pancreatitis. Patient was discharged with complete resolution of the extrabdominal disease.
  
  
  CONCLUSIONS
  In literature only 64 cases of PPP syndrome have been reported. Abdominal symptoms do not often appear at presentation and diagnosis may be delayed. Panniculitis develope in any part of the body but especially on the distal parts of the lower extremities, around the ankles and pretibial regions of the legs. Between osteo-articular manifestations polyarthritis is the most common one, although oligoarthritis, and monoarthritis in have been reported.
  
  
  CONCLUSIONS
  PPP syndrome is a rare disease with a high mortality rate. A timely diagnosis and an aggressive treatment may improve the prognosis of this condition.
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain. Electronic address: valenpeglio@gmail.com.
authors.forename: Valentina
authors.initials: V
authors.lastname: Ferri
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Benedetto
authors.initials: B
authors.lastname: Ielpo
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Hipolito
authors.initials: H
authors.lastname: Duran
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Diaz
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Isabel
authors.initials: I
authors.lastname: Fabra
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Caruso
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Luisi
authors.initials: L
authors.lastname: Malave
authors.affiliation: Division of Anatomic Pathology, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Plaza
authors.affiliation: Division of Reumathology Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Silvia
authors.initials: S
authors.lastname: Rodriguez
authors.affiliation: Diviasion of Nuclear Medicin, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Lina
authors.initials: L
authors.lastname: Garcia
authors.affiliation: Division or Radiology, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Virginia
authors.initials: V
authors.lastname: Perez
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Yolanda
authors.initials: Y
authors.lastname: Quijano
authors.affiliation: Division of General Surgery, Sanchinarro Hospital, San Pablo University, Madrid, Spain.
authors.forename: Emilio
authors.initials: E
authors.lastname: Vicente
keywords: Case report; Pancreatic cancer; Pancreatits; Panniculitis; Poliarthritis