Pancreatic Panniculitis Associated with Acinar Cell Carcinoma of the Pancreas: A Case Report

Abstract

Pancreatic panniculitis is a rare type of disorder associated with pancreatic diseases. We describe here a case of 54-year-old man who was admitted to the Department of Dermatology with the diagnosis of erythema nodosum. The patient presented with a 9-month history of painful erythematous nodules on the extremities, joint pain and swelling, and weight loss. A highly elevated level of pancreatic lipase was found on the laboratory examinations. The biopsy specimens from the skin lesions showed subcutaneous fat necrosis. Abdominal computed tomography (CT) revealed a large mass with central necrosis in the body and tail of the pancreas. Distal pancreatectomy, splenectomy and partial transverse colectomy were successfully performed on day 17 of the hospitalization. The histopathologic findings supported the diagnosis of acinar cell carcinoma of the pancreas (ACCP). Postoperatively, the level of serum lipase returned to normal, and the skin lesions and joint manifestations gradually regressed. However, the swelling did not significantly resolve in the left knee. In view of the non-specific clinical presentation of this disease, clinicians should be alert and have a high index of suspicion for pancreatic panniculitis.

Polyarthritis and Pancreatic Panniculitis Associated With Pancreatic Carcinoma

“…2 In addition to our case, we identified in the English literature 30 well-documented cases of complete PPP syndrome associated with pancreatic carcinoma. 16,[20][21][22][23][24][25][26][28][29][30][31][32][33][34][35][36][37][38] These patients were mostly elderly men (87%) with a mean age at diagnosis of 61.2 ± 13 years (range, 20-88 years). Acinar cell carcinoma, in 58% of the cases, was the most common pancreatic neoplasm, whereas metastatic disease was documented in 21 patients.…”

“…In cases associated with acute or chronic pancreatitis, the mortality rate was as high as 24%, 2 and when associated with pancreatic carcinoma, it was nearly 74%. [20][21][22][23][24][25][26][28][29][30][31][32][33][34][35][36][37][38] Subcutaneous nodules may be the presenting symptom of an otherwise occult pancreatic disease. In fact, Hughes et al 17 reported that panniculitis was the presenting symptom in 65% of patients with pancreatic carcinoma.…”

“…[1][2][3][4] This triad is known as the pancreatitis, panniculitis, and polyarthritis (PPP) syndrome and has been described mainly in patients with acute or chronic pancreatitis 2,4-18 and pancreatic carcinoma, usually acinar cell carcinoma. 11,17,[19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38] Other pancreatic disorders have rarely been associated with pancreatic panniculitis and/or polyarthritis including pancreatic pseudocyst, 2,12 ischemic pancreatic disease, 39 abdominal trauma, 40 and pancreas divisum. 41 The absence of abdominal symptoms in some patients may lead to misdiagnosis of pancreatic disease with a worsening of prognosis of this condition.…”

Spontaneous rupture of a pancreatic acinar cell carcinoma presenting as an acute abdomen

“…Jaundice is an infrequent finding (12%) and complications related to lipase release (panniculitis) are common. 2 Abdominal pain is reported to be present in 32% and nausea and vomiting in 23% of patients. 3 To the best of our knowledge, this is the first described case of spontaneous rupture of ACC that presented as an acute abdomen.…”

Xanthogranulomatous Cholecystitis-induced Pancreatic Panniculitis

“…Pancreatic panniculitis rarely (2-3%) accompanies pancreatic disease; therefore, diagnosing the disease can sometimes be difficult (2). Physicians should include pancreatic panniculitis in the differential diagnosis of erythema nodosum.…”