Intramedullary fat necrosis, polyarthritis and panniculitis with pancreatic tumor: a case report

Abstract

Acute polyarthritis can occur in non-rheumatic systemic illnesses, presenting a diagnostic dilemma. We present an extremely rare case presenting as acute polyarthritis, panniculitis and medullary fat necrosis with underlying pancreatic pathology. This case report describes a young woman presenting with panniculits, pancreatic tumour, polyarthritis and intra-osseus fat necrosis with a fatal outcome. The medical fraternity needs to be aware of this potentially fatal albeit rare musculoskeletal complication secondary to a pancreatic pathology.

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome: MRI features of intraosseous fat necrosis involving the feet and knees

“…The underlying pancreatic disease is either acute or chronic pancreatitis [1]. Previous reports have shown that a pancreatic mass, such as a pseudopapillary pancreatic tumor, a malignancy (composed of acinar cell types), or a pancreatic pseudocyst, can also cause PPP syndrome [4][5][6][18][19][20]. Our patient had acute pancreatitis, subcutaneous fat necrosis, and polyarthritis, including medullary fat necrosis.…”

“…However, polyarthritis more rarely presents as a complication of pancreatic disease. Therefore, PPP syndrome is extremely rare and presents as a clinical triad of pancreatitis, panniculitis, and polyarthritis [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17]. The underlying pancreatic disease is either acute or chronic pancreatitis [1].…”

“…The joint symptoms are transient in most cases [1]. Another indication of musculoskeletal involvement in PPP syndrome can present as intraosseous fat necrosis [1,4,10,11,13,14].…”

“…This triad is referred to pancreatitis, panniculitis, and polyarthritis (PPP) syndrome in the literature [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17]. Despite the rarity of PPP syndrome, it is important to understand and be familiar with its indicators because of the possibility of misdiagnosis or a delayed diagnosis due to absent or mild abdominal symptoms [1,4,7,12,16,17].…”

Skin lesions, elevated serum lipase level and abnormal bone scintigraphy

“…Lipase released into the bloodstream in large amounts (and also amylase and trypsin in some cases, although in our patient the amylase level was normal) is responsible for the digestion of subcutaneous fat (panniculitis) and can affect articular surfaces of joints (arthritis) as well as the intramedullary fat of bones [2]. Our patient presented with all three manifestations of the disease.…”