Clinical Analysis of Pancreatitis, Panniculitis, and Polyarthritis Syndrome_West China Medical Journal

Authors：

 LINHui ¹ , SHIXiao-jun ² , WANGYing ¹ , TANGHong-hu ¹ ,  LIUYi ¹

1. Department of Rheumatology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;
2. Department of Orthopedic Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

LIUYi, Email: yi2006liu@163.com

Keywords：

Pancreatitis; Pancreatic disease; Panniculitis; Arthritis

DOI：

10.7507/1002-0179.20140322

Video：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

Objective To investigate the clinical characteristics of pancreatitis, panniculitis, and polyarthritis syndrome (PPP syndrome). Methods A patient with PPP syndrome in July 2013 was reported. The clinical features of 33 cases of PPP syndrome at home and abroad were reviewed. Results Our patient with panniculitis and arthritis as the first manifestation had few abdominal symptoms despite high serum levels of pancreatic enzymes. In the 34 PPP cases, 70.6% (24/34) had absent or mild abdominal symptoms, easily leading to misdiagnosis. Multi-joint involvement was more common, and in almost 1/3 of the patients, joint symptoms with poor NSAIDs and/or hormonal responses predated the identification of pancreatic disease. Panniculitis biopsy found characteristic "ghost cells". Ten patients died of complications of pancreatic disease (including 2 cases of cancer), and the mortality rate was as high as 29.4% (10/34). Conclusion PPP syndrome with mild abdominal symptoms and high mortality should be taken for the treatment of pancreatic disease, to prevent misdiagnosis and mistreatment.

Citation： LINHui, SHIXiao-jun, WANGYing, TANGHong-hu, LIUYi. Clinical Analysis of Pancreatitis, Panniculitis, and Polyarthritis Syndrome. West China Medical Journal, 2014, 29(6): 1050-1054. doi: 10.7507/1002-0179.20140322 Copy

1.	Moro M, Moletta L, Blandamura S, et al. Acinar cell carcinoma of the pancreas associated with subcutaneous panniculitis[J]. JOP, 2011, 12(3): 292-296.
2.	Corazza M, Salmi R, Strumia R. Pancreatic panniculitis as a first sign of liver carcinoma[J]. Acta Derm Venereol, 2003, 83(3): 230-231.
3.	Boswell SH, Baylin GJ. Metastatic fat necrosis and lytic bone lesions in a patient with painless acute pancreatitis[J]. Radiology, 1973, 106(1): 85-86.
4.	Simkin PA, Brunzell JD, Wisner D, et al. Free fatty acids in the pancreatitic arthritis syndrome[J]. Arthritis Rheum, 1983, 26(2): 127-132.
5.	Wilson HA, Askari AD, Neiderhiser DH, et al. Pancreatitis with arthropathy and subcutaneous fat necrosis: evidence for the pathogenicity of lipolytic enzymes[J]. Arthritis Rheum, 1983, 26(2): 121-126.
6.	Baron M, Paltiel H, Lander P. Aseptic necrosis of the talus and calcaneal insufficiency fractures in a patient with pancreatitis, subcutaneous fat necrosis, and arthritis[J]. Arthritis Rheum, 1984, 27(11): 1309-1313.
7.	Saag KG, Niemann TH, Warner CA, et al. Subcutaneous pancreatic fat necrosis associated with acute arthritis[J]. J Rheumatol, 1992, 19(4): 630-632.
8.	Haller J, Greenway G, Rennick D, et al. Intraosseous fat necrosis associated with acute pancreatitis: Mr imaging[J]. Radiology, 1989, 173(1): 193-195.
9.	Watts RA, Kelly S, Hacking JC, et al. Fat necrosis. An unusual cause of polyarthritis[J]. J Rheumatol, 1993, 20(8): 1432-1435.
10.	Ferrari R, Wendelboe M, Ford PM, et al. Pancreatitis arthritis with periarticular fat necrosis[J]. J Rheumatol, 1993, 20(8): 1436-1437.
11.	L'hirondel JL, Fournier L, Fretille A, et al. Intraosseous fat necrosis and metaphyseal osteonecrosis in a patient with chronic pancreatitis: Mr imaging and CT scanning[J]. Clin Exp Rheumatol, 1994, 12(2): 191-194.
12.	Rodriguez M, Lopez GL, Prieto P, et al. Massive subcutaneous and intraosseous fat necrosis associated with pancreatitis. Natural evolution of the radiographic picture[J]. Clin Rheumatol, 1997, 16(2): 199-203.
13.	Francombe J, Kingsnorth AN, Panniculitis TE. Arthritis and pancreatitis[J]. Br J Rheumatol, 1995, 34(7): 680-683.
14.	Shbeeb MI, Duffy J, Bjornsson J, et al. Subcutaneous fat necrosis and polyarthritis associated with pancreatic disease[J]. Arthritis Rheum, 1996, 39(11): 1922-1925.
15.	Ordás CC, Jirout CF, Cerrato RM, et al. Nodular lesions and arthritis associated with chronic alcoholism[J]. Rev Clin Esp, 1998, 198(9): 619-620.
16.	Lopez A, Garcia-Estan J, Marras C, et al. Pancreatitis associated with pleural-mediastinal pseudocyst, panniculitis and polyarthritis[J]. Clin Rheumatol, 1998, 17(4): 335-339.
17.	Kotilainen P, Saario R, Mattila K, et al. Intraosseous fat necrosis simulating septic arthritis and osteomyelitis in a patient with chronic pancreatitis[J]. Arch Orthop Trauma Surg, 1998, 118(3): 174-175.
18.	Karasick D, Schweitzer ME. Case 4: intraosseous fat necrosis associated with pancreatitis[J]. Radiology, 1998, 209(2): 521-524.
19.	Mourad FH, Hannoush HM, Bahlawan M, et al. Panniculitis and arthritis as the presenting manifestation of chronic pancreatitis[J]. J Clin Gastroenterol, 2001, 32(3): 259-261.
20.	Preiss JC, Faiss S, Loddenkemper C, et al. Pancreatic panniculitis in an 88-year-old man with neuroendocrine carcinoma[J]. Digestion, 2002, 66(3): 193-196.
21.	Morita O, Ogose A, Hotta T, et al. Pathological fractures due to intraosseous fat necrosis associated with pancreatitis[J]. Rheumatology (Oxford), 2003, 42(2): 394-396.
22.	Menon P, Kulshreshta R. Pancreatitis with panniculitis and arthritis: a rare association[J]. Pediatr Surg Int, 2004, 20(2): 161-162.
23.	Price-Forbes A, Filer A, Udeshi UL, et al. Progression of imaging in pancreatitis panniculitis polyarthritis (PPP) syndrome[J]. Scand J Rheumatol, 2006, 35(1): 72-74.
24.	Narváez J, Bianchi MM, Santo P, et al. Pancreatitis, panniculitis, and polyarthritis[J]. Semin Arthritis Rheum, 2010, 39(5): 417-423.
25.	Jose T, Biju IK, Kumar A, et al. Pancreatitis, polyarthritis, panniculitis syndrome (PPP syndrome) plus prolonged pyrexia-a rare presentation of chronic pancreatitis[J]. Indian J Gastroenterol, 2009, 28(5): 186-188.
26.	Borowicz J, Morrison M, Hogan D, et al. Subcutaneous fat necrosis/panniculitis and polyarthritis associated with acinar cell carcinoma of the pancreas: a rare presentation of pancreatic, panniculitis and polyarthritis syndrome[J]. J Drugs Dermatol, 2010, 9(9): 1145-1150.
27.	Mustafa KN, Hadidy A, Shoumaf M, et al. Polyarthritis with chondronecrosis associated with osteonecrosis, panniculitis and pancreatitis[J]. Rheumatol Int, 2010, 30(9): 1239-1242.
28.	Fraisse T, Boutet O, Tron AM, et al. Pancreatitis, panniculitis, polyarthritis syndrome: an unusual cause of destructive polyarthritis[J]. Joint Bone Spine, 2010, 77(6): 617-618.
29.	Harris MD, Bucobo JC, Buscaglia JM. Pancreatitis, panniculitis, polyarthritis syndrome successfully treated with EUS-guided cyst-gastrostomy[J]. Gastrointest Endosc, 2010, 72(2): 456-458.
30.	Kuwatani M, Kawakami H, Yamada Y. Osteonecrosis and panniculitis as life-threatening signs[J]. Clin Gastroenterol Hepatol, 2010, 8(5): e52-e53.
31.	钱龙, 李向培, 杨国俊. 脂膜炎关节炎和胰腺炎三联征一例[J]. 中华风湿病学杂志, 2004, 8(1): 61-62.
32.	Garcia-Romero D, Vanaclocha F. Pancreatic panniculitis[J]. Dermatol Clin, 2008, 26(4): 465-470.
33.	Requena L, Sitthinamsuwan P, Santonja C, et al. Cutaneous and mucosal mucormycosis mimicking pancreatic panniculitis and gouty panniculitis[J]. J Am Acad Dermatol, 2012, 66(6): 975-984.
34.	Rongioletti F, Caputo V. Pancreatic panniculitis[J]. G Ital Dermatol Venereol, 2013, 148(4): 419-425.

1. Moro M, Moletta L, Blandamura S, et al. Acinar cell carcinoma of the pancreas associated with subcutaneous panniculitis[J]. JOP, 2011, 12(3): 292-296.
2. Corazza M, Salmi R, Strumia R. Pancreatic panniculitis as a first sign of liver carcinoma[J]. Acta Derm Venereol, 2003, 83(3): 230-231.
3. Boswell SH, Baylin GJ. Metastatic fat necrosis and lytic bone lesions in a patient with painless acute pancreatitis[J]. Radiology, 1973, 106(1): 85-86.
4. Simkin PA, Brunzell JD, Wisner D, et al. Free fatty acids in the pancreatitic arthritis syndrome[J]. Arthritis Rheum, 1983, 26(2): 127-132.
5. Wilson HA, Askari AD, Neiderhiser DH, et al. Pancreatitis with arthropathy and subcutaneous fat necrosis: evidence for the pathogenicity of lipolytic enzymes[J]. Arthritis Rheum, 1983, 26(2): 121-126.
6. Baron M, Paltiel H, Lander P. Aseptic necrosis of the talus and calcaneal insufficiency fractures in a patient with pancreatitis, subcutaneous fat necrosis, and arthritis[J]. Arthritis Rheum, 1984, 27(11): 1309-1313.
7. Saag KG, Niemann TH, Warner CA, et al. Subcutaneous pancreatic fat necrosis associated with acute arthritis[J]. J Rheumatol, 1992, 19(4): 630-632.
8. Haller J, Greenway G, Rennick D, et al. Intraosseous fat necrosis associated with acute pancreatitis: Mr imaging[J]. Radiology, 1989, 173(1): 193-195.
9. Watts RA, Kelly S, Hacking JC, et al. Fat necrosis. An unusual cause of polyarthritis[J]. J Rheumatol, 1993, 20(8): 1432-1435.
10. Ferrari R, Wendelboe M, Ford PM, et al. Pancreatitis arthritis with periarticular fat necrosis[J]. J Rheumatol, 1993, 20(8): 1436-1437.
11. L'hirondel JL, Fournier L, Fretille A, et al. Intraosseous fat necrosis and metaphyseal osteonecrosis in a patient with chronic pancreatitis: Mr imaging and CT scanning[J]. Clin Exp Rheumatol, 1994, 12(2): 191-194.
12. Rodriguez M, Lopez GL, Prieto P, et al. Massive subcutaneous and intraosseous fat necrosis associated with pancreatitis. Natural evolution of the radiographic picture[J]. Clin Rheumatol, 1997, 16(2): 199-203.
13. Francombe J, Kingsnorth AN, Panniculitis TE. Arthritis and pancreatitis[J]. Br J Rheumatol, 1995, 34(7): 680-683.
14. Shbeeb MI, Duffy J, Bjornsson J, et al. Subcutaneous fat necrosis and polyarthritis associated with pancreatic disease[J]. Arthritis Rheum, 1996, 39(11): 1922-1925.
15. Ordás CC, Jirout CF, Cerrato RM, et al. Nodular lesions and arthritis associated with chronic alcoholism[J]. Rev Clin Esp, 1998, 198(9): 619-620.
16. Lopez A, Garcia-Estan J, Marras C, et al. Pancreatitis associated with pleural-mediastinal pseudocyst, panniculitis and polyarthritis[J]. Clin Rheumatol, 1998, 17(4): 335-339.
17. Kotilainen P, Saario R, Mattila K, et al. Intraosseous fat necrosis simulating septic arthritis and osteomyelitis in a patient with chronic pancreatitis[J]. Arch Orthop Trauma Surg, 1998, 118(3): 174-175.
18. Karasick D, Schweitzer ME. Case 4: intraosseous fat necrosis associated with pancreatitis[J]. Radiology, 1998, 209(2): 521-524.
19. Mourad FH, Hannoush HM, Bahlawan M, et al. Panniculitis and arthritis as the presenting manifestation of chronic pancreatitis[J]. J Clin Gastroenterol, 2001, 32(3): 259-261.
20. Preiss JC, Faiss S, Loddenkemper C, et al. Pancreatic panniculitis in an 88-year-old man with neuroendocrine carcinoma[J]. Digestion, 2002, 66(3): 193-196.
21. Morita O, Ogose A, Hotta T, et al. Pathological fractures due to intraosseous fat necrosis associated with pancreatitis[J]. Rheumatology (Oxford), 2003, 42(2): 394-396.
22. Menon P, Kulshreshta R. Pancreatitis with panniculitis and arthritis: a rare association[J]. Pediatr Surg Int, 2004, 20(2): 161-162.
23. Price-Forbes A, Filer A, Udeshi UL, et al. Progression of imaging in pancreatitis panniculitis polyarthritis (PPP) syndrome[J]. Scand J Rheumatol, 2006, 35(1): 72-74.
24. Narváez J, Bianchi MM, Santo P, et al. Pancreatitis, panniculitis, and polyarthritis[J]. Semin Arthritis Rheum, 2010, 39(5): 417-423.
25. Jose T, Biju IK, Kumar A, et al. Pancreatitis, polyarthritis, panniculitis syndrome (PPP syndrome) plus prolonged pyrexia-a rare presentation of chronic pancreatitis[J]. Indian J Gastroenterol, 2009, 28(5): 186-188.
26. Borowicz J, Morrison M, Hogan D, et al. Subcutaneous fat necrosis/panniculitis and polyarthritis associated with acinar cell carcinoma of the pancreas: a rare presentation of pancreatic, panniculitis and polyarthritis syndrome[J]. J Drugs Dermatol, 2010, 9(9): 1145-1150.
27. Mustafa KN, Hadidy A, Shoumaf M, et al. Polyarthritis with chondronecrosis associated with osteonecrosis, panniculitis and pancreatitis[J]. Rheumatol Int, 2010, 30(9): 1239-1242.
28. Fraisse T, Boutet O, Tron AM, et al. Pancreatitis, panniculitis, polyarthritis syndrome: an unusual cause of destructive polyarthritis[J]. Joint Bone Spine, 2010, 77(6): 617-618.
29. Harris MD, Bucobo JC, Buscaglia JM. Pancreatitis, panniculitis, polyarthritis syndrome successfully treated with EUS-guided cyst-gastrostomy[J]. Gastrointest Endosc, 2010, 72(2): 456-458.
30. Kuwatani M, Kawakami H, Yamada Y. Osteonecrosis and panniculitis as life-threatening signs[J]. Clin Gastroenterol Hepatol, 2010, 8(5): e52-e53.
31. 钱龙, 李向培, 杨国俊. 脂膜炎关节炎和胰腺炎三联征一例[J]. 中华风湿病学杂志, 2004, 8(1): 61-62.
32. Garcia-Romero D, Vanaclocha F. Pancreatic panniculitis[J]. Dermatol Clin, 2008, 26(4): 465-470.
33. Requena L, Sitthinamsuwan P, Santonja C, et al. Cutaneous and mucosal mucormycosis mimicking pancreatic panniculitis and gouty panniculitis[J]. J Am Acad Dermatol, 2012, 66(6): 975-984.
34. Rongioletti F, Caputo V. Pancreatic panniculitis[J]. G Ital Dermatol Venereol, 2013, 148(4): 419-425.

Previous Article
急性百草枯中毒致急性肺损伤发病机制的研究进展
Next Article
超声在纵膈巨大囊肿麻醉中的应用一例

West China Medical Journal

Clinical Analysis of Pancreatitis, Panniculitis, and Polyarthritis Syndrome

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content