the association of pancreatic disease with fat necrosis at distant foci was first described by chiari in 1883 ( 1 ) .
the most common pancreatic disorders associated with pancreatic panniculitis are acute or chronic pancreatitis , especially the alcohol - related types , and pancreatic carcinoma .
other pancreatic disorders have been infrequently reported to be associated with pancreatic panniculitis , and these include post - traumatic pancreatitis , pancreatic pseudocyst , pancreas divisum , and hemophagocytic syndrome ( 2 ) . to the best of our knowledge ,
one case was associated with pancreatic adenocarcinoma , and the other two were associated with acute and chronic pancreatitis , respectively ( 1 , 3 , 4 ) .
we report here on a case of pancreatic panniculitis associated with acute pancreatitis that had a fatal outcome .
physicians should be aware of this disease entity because this rare cutaneous manifestation may be associated with major morbidity and significant mortality .
a 50-yr - old man with a history of alcohol abuse presented with increasing fatigue , generalized weakness , decreased appetite , and abdominal distension and discomfort for the past 2 weeks .
he was admitted to the department of internal medicine under the impression of acute pancreatitis , and he was referred to the department of dermatology for the multiple painful subcutaneous nodules on his legs , which had suddenly developed 3 weeks before ( fig .
his hemoglobin value was 11.4 g / dl ( reference range : 13 - 18 g / dl ) , the total count of white blood cells was 31,300/l ( reference range : 4,000 - 10,000/l ) with 90.7% segment neutrophils , but the coagulation profiles and platelet counts were normal .
the serum amylase was 1,909 u / l ( reference range : 20 - 120 u / l ) , and the lipase was 2,306 u / l ( reference range : 5 - 51
u / l ) , an alanine aminotransferase level of 24 u / l ( reference range : 7 - 40
u / l ) , and a lactate dehydrogenase level of 665 u / l ( reference range : 200 - 400
the fasting glucose level was 133.9 mg / dl ( reference range of 70 - 110 mg / dl ) and the electrolytes were unbalanced .
the calcium level was 7 mg / dl ( reference range : 8.6 - 10 mg / dl ) , and the sodium level was 125
the blood urea nitrogen and creatinine were 40.1 mg / dl and 2.1 mg / dl , respectively .
after 48 hr , the blood urea nitrogen was increased to 62.7 mg / dl after intravenous fluid administration .
as his leukocytosis , elevated serum ldh at admission , and hypocalcemia , hypoalbuminemia , increase in blood urea nitrogen during initial 48 hr were poor prognostic factors in ranson criteria , increased risk of complications was predicted .
the chest radiograpy showed mild pleural effusion . on the abdominal computed tomography scan and magnetic resonance ( mr ) imaging taken on the second day of admission , a swollen pancreas with an dilated pancreatic duct , a loculated fluid collection in the left anterior perirenal space , multiple hepatic cysts , and massive ascites
u / l , the serum - ascites albumin gradient was calculated to be 1.07 , and there was no evidence of malignancy .
a skin biopsy performed on the 4th day of admission from the nodule on the left lower leg showed a diffuse subcutaneous fat necrosis and ghost - like cells with thick shadowy walls and no nuclei .
there was a fine granular basophilic material deposited within and around the necrotic fat cells ( fig .
after 10 days of intensive medical care for the pancreatic disease , the patient 's condition began to worsen ; he and his family began to refuse any further treatment . despite a strong warning by physicians
the pathogenesis of pancreatic panniculitis is still unknown , but the released pancreatic enzymes such as trypsin may increase the permeability of the microcirculation .
lipase or amylase is then involved in the process of fat degradation , which results in the liberation of free fatty acids that combine with calcium to form soap ( 2 ) .
although elevation of the pancreatic enzymes is common in pancreatitis patients , pancreatic panniculitis is a very rare malady .
mullin et al . ( 5 ) identified only one case in a retrospective review of 893 patients who had suffered with pancreatic disease from various causes .
furthermore , well documented cases of fat necrosis with normal serum lipase levels have also been described ( 6 ) .
these reports , suggest that there would be some other factors that allow the pancreatic enzymes to escape from the circulation and act on the subcutaneous fat .
zellman ( 7 ) suggested that some damage to the blood vessels via inflammation , edema , or altered immunity may act as the initiating factor .
as for the two cases in the korean literature , one case was associated with chronic pancreatisis , in which the cutaneous lesions had occurred 3 months later than the other systemic symptoms , and they involuted as the underlying pancreatitis was ameliorated ( 4 ) .
the other case was associated with acute pancreatitis with pancreas divisum , in which his skin lesions had appeared 2 - 3 days after his admission due to acute pancreatitis ; the skin lesions also resolved as the underlying pancratitis subsided with conservative treatment ( 1 ) . on the contrary , in our patient , the skin lesions preceded the other systemic symptoms .
although the underlying pancreatic pathologic conditions can vary , the clinical features of pancreatic panniculitis are similar .
the legs were the most commonly affected area , but the lesions can also occur on the arms , thighs , and trunk .
the lesions usually began as erythematous or red - brown subcutaneous nodules with a tendency to show central softening . in the mild form
if the fat necrosis is severe , individual nodules may break down and extrude necrotic material ( 8) . although patients with other panniculitides such as erythema nodosum , erythema induratum , lupus panniculitis , weber - christian pannicultitis , or alpha-1 antitrypsin deficiency - associated panniculitis can have similar clinical lesions , the diagnosis of pancreatic panniculitis is suggested by the presence of pancreatic disease and this
the pathognomonic findings are collections of ' ghost cells ' and anucleated adipocytes containing intracytoplasmic fine basophilic granular material from the saponification of fat .
the resistance of the fat cell membrane to lipase produces the shadowy walls and the fatty acids combine with calcium to form calcium soap ( 10 ) .
sometimes conservative care can ameliorate the pancreatitis , and this then results in the resolution of the skin lesions . in isolated cases , surgical correction of anatomic ductal anomaly or pancreatic pseudocyst , or
the removal of gallstones has resulted in complete resolution ( 8) . however , disseminated fat necrosis is associated with major morbidity and mortality . in a review of 27 patients with pancreatic panniculitis , all 8 patients with pancreatic carcinoma and 42% of the 19 patients with pancreatitis died of their disease , as was seen in our case ( 9 ) .
pancreatic panniculitis is a pathognomic finding of pancreatic disease , and as in our case , the cutaneous lesions may precede the usual manifestations of underlying pancreatic disease by several weeks to months ( 11 ) .
 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 . 