Pancreatic Panniculitis
Section snippets
Pathogenesis
The exact pathogenic mechanism of pancreatic panniculitis is still unclear, but release of pancreatic enzymes, such as lipase, phosphorilase, trypsin, and amylase, may be involved. The activation mechanism of pancreatic enzyme precursors into activated forms is not clear.12 It is tempting to speculate that trypsin may increase the permeability of the microcirculation within lymphatic vessels,10 allowing the enzymes, such a lipase or amylase, to enter into the fat lobules and hydrolyze neutral
Clinical manifestations
Clinically, pancreatic panniculitis presents with ill-defined, tender, edematous, erythematous, or red-brown nodules that may spontaneously ulcerate and drain an oily brown, sterile, and viscous substance that results from liquefaction necrosis of adipocytes. These lesions are usually located on the distal parts of the lower extremities (Fig. 1), around the ankles and knees, although nodules can spread over the thighs, buttocks, arms, abdomen, chest, and scalp. Cases of pancreatic panniculitis
Associations
The most common underlying pancreatic disorders associated with pancreatic panniculitis are acute10 and chronic pancreatitis,24, 25 which usually results from alcohol abuse,10 trauma,26, 27or cholelithiasis,28 but it has also been described as a complication of pancreatic carcinoma,29 usually acinar cell carcinoma,7, 14, 23, 30, 31, 32, 33, 34 and less frequently islet cell carcinoma1, 23, 35, 36, 37, 38, 39, 40 and acinar cell cystadenocarcinoma.15 Rarely, other pancreatic disorders are
Histopathology
The histopathology of pancreatic panniculitis is patognomonic, where the main histopathologic feature is a predominantly lobular panniculitis without vasculitis.1, 10 Biopsy specimens from fully developed lesions of pancreatic panniculitis show a characteristic coagulative necrosis of the adipocytes, which leads to “ghost adipocytes.” Ghost adipocytes are cells that have lost their nucleus, and have a thick shadowy wall with a fine basophilic granular or homogeneous material within their
Laboratory findings
Serum levels of amylase, lipase, or trypsin are usually elevated in pancreatic panniculitis, although not in all cases. In addition, it is common that one enzyme is within normal levels, whereas others are increased,4 so panniculitis does not correlate with enzyme levels.50 In rare instances, patients may have high serum levels of pancreatic lipase and no evidence of underlying pancreatic disease.18 It is necessary, however, to perform serum levels of lipase, amylase, and trypsin when a
Differential diagnosis
Clinically, the nodules of pancreatic panniculitis can mimic other forms of panniculitis, such as erythema nodosum, erythema induratum, α1-antitrypsin deficiency panniculitis, infectious panniculitis, or subcutaneous metastasis.1, 11, 13 The propensity to ulceration and discharge of an oily material argues against these diseases.13
The histopathologic differential diagnosis includes erythema nodosum, erythema induratum, traumatic panniculitis, infectious panniculitis, α1-antitrypsin deficiency
Treatment
Treatment of pancreatic panniculitis is primarily supportive and should be directed to the underlying pancreatic disease. In patients with acute pancreatitis, the nodules disappear when the acute inflammatory phase is over.14 In cases of chronic pancreatitis, cholecystectomy and gallstone pancreatic duct removal could resolve the panniculitis.4 In the case of pancreatic fistula or cyst, a pancreatic duct stent can be used to relieve obstruction.52 Biliary bypass surgery can be useful if simple
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