Fatal Pancreatic Panniculitis Associated with Acute Pancreatitis: A Case Report

Abstract

Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occurs in the setting of pancreatic diseases; these diseases include acute and chronic pancreatitis, pancreatic carcinoma, pseudocyst, and other pancreatic diseases. This malady is manifested as tender erythematous nodules on the legs, buttock, or trunk. Histopathologically, it shows the pathognomonic findings of focal subcutaneous fat necrosis and ghost-like anucleated cells with a thick shadowy wall. We herein report a case of fatal pancreatic panniculitis that was associated with acute pancreatitis in a 50-yr-old man. He presented with a 3-week history of multiple tender skin nodules, abdominal pain and distension. Laboratory and radiologic findings revealed acute pancreatitis, and skin biopsy showed pancreatic panniculitis. Despite intensive medical care, he died of multi-organ failure 3 weeks after presentation.

Paniculite pancreática como a primeira manifestação de doença visceral: relato de caso

“…6 Cases of pancreatic panniculitis with normal enzyme levels have been reported. 2,5,6 These reports and the discrepancy between the relative frequency of the disease and the frequency of pancreatic panniculitis suggest the involvement of an additional etiological factor. One hypothesis is that these patients are unable to degrade pancreatic enzymes, probably due to inherited enzyme deficiencies such as alpha 1-antitrypsin (AAT).…”

“…However, pancreatic pseudocyst, post-traumatic pancreatitis, pancreas divisum and pancreatic vascular fistulas have also been reported. 1,2,6,7 The pathogenesis is unknown, but it is assumed that the release of pancreatic enzymes such as trypsin may increase microcirculation permeability, resulting in lipase and amylase causing the subcutaneous fat necrosis observed in the lesions. 6 Cases of pancreatic panniculitis with normal enzyme levels have been reported.…”

“…1,2,6,7 The pathogenesis is unknown, but it is assumed that the release of pancreatic enzymes such as trypsin may increase microcirculation permeability, resulting in lipase and amylase causing the subcutaneous fat necrosis observed in the lesions. 6 Cases of pancreatic panniculitis with normal enzyme levels have been reported. 2,5,6 These reports and the discrepancy between the relative frequency of the disease and the frequency of pancreatic panniculitis suggest the involvement of an additional etiological factor.…”

“…6 Histologically, pancreatic panniculitis is a predominantly lobular panniculitis. Initial lesions are characterized by lymphoplasmacytic infiltration along the fibrous septum around subcutaneous fat lobules and dermal blood vessels.…”

“…It is suspected that it is caused by the release of trypsin, lipase, amylase and phospholipase in the peripheral circulation. 1,2,6 The case report below refers to chronic pancreatitis which, contrary to the normal pattern of progression, at first presented clinically as pancreatic panniculitis.…”