Fatal case of pancreatic panniculitis caused by occult neuroendocrine tumor in the corresponding organ: A case report and review of the published work

Abstract

Pancreatic panniculitis (PP) is a rare clinical variant of subcutaneous fat necrosis, developing in patients with a variety of pancreatic diseases such as acute or chronic pancreatitis, tumors and cysts. The tumor‐associated PP represents a noteworthy skin manifestation of underlying internal malignancies, also known as dermadrome. Among causative pancreatic tumors, acinar cell carcinoma is the most frequent malignancy; however, little is known about how the origin of tumor cells and progression stage of pancreatic tumors potentially contribute to the establishment of panniculitis. Here, we present a 69‐year‐old Japanese male case of clinically aggressive PP on the bilateral legs, whose skin lesions developed prior to the diagnosis of occult pancreatic tumor and liver metastasis. Moreover, the immunopathology of the pancreatic lesion revealed neuroendocrine tumor (NET), a rare pathological variant. Skin lesions immediately spread to the upper limbs with extensive ulcerations and necrosis, accompanied by high levels of serum lipase and elastase, but not with other pancreatic enzymes. He died 2 months after the initial development of the skin lesion due to rapid deterioration of general condition. We reviewed 14 cases, including ours, of PP with NET in the pancreas thus far reported, to identify the clinicopathological characteristics regarding to what extent this rare complication could reflect the clinical course of pancreatic tumors and overall prognosis. Our published work review found that the disease has a significant male predominance (male : female, 13:1) and cases with occult pancreatic tumors died within 4 months after the development of their skin lesions. Our case was the poorest prognostic outcome. This report emphasizes that dermatologists should recognize PP with NET, reflecting a fatal prognosis, and to make a prompt diagnosis.

A case of pancreatitis‐panniculitis‐polyarthritis syndrome due to pancreatic neuroendocrine tumor

“…1 However, it is extraordinarily associated with pancreatic neuroendocrine tumor (PanNET), with only three of 59 PPP syndrome cases and 14 pancreatic panniculitis (PP) cases caused by PanNET. 1,3 We report a middle-aged woman with PanNETinduced PPP syndrome.…”

“…4 The average patient reads at grade level 7, and nearly 20% of Americans have difficulty comprehending reading grade level 4 text. [3][4][5] This indicates that currently available online resources for PV and BP are on average at least six reading grades higher than recommended and at least five grades higher than the average patient's reading ability. Remarkably, we found that resources written by medical doctors, and especially by dermatologists, had higher ARGL than those written by non-medical doctors.Limitations of our study included the cross-sectional design and possible multi-rater variability across the different validated readability measures.…”

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Approximately 80 million Americans have limited health literacy, and lower health literacy has been associated with both poorer access to care and health outcomes. 3 The American Medical Association and the National Health Institutes recommend that the readability of patient education materials be at reading grade level 6 or lower. 4 The average patient reads at grade level 7, and nearly 20% of Americans have difficulty comprehending reading grade level 4 text.

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“…Empirical broad-spectrum antibiotics were prescribed for 10 days due to intermittent pyrexia (39.3°C) with negative blood The underlying mechanism of PPP syndrome could be involved in subcutaneous adiponecrosis and intramedullary lipolysis caused by pancreatic enzymes, especially lipase. 1,3 PP can precede, coincide, or succeed pancreatic conditions and often presents as tender, red-brown nodules on the lower limbs. 2 The nodules can ulcerate and release a brown lipid-rich material due to fat necrosis.…”

“…2 The nodules can ulcerate and release a brown lipid-rich material due to fat necrosis. [1][2][3] The differential diagnosis includes erythema nodosum, erythema induratum, cutaneous polyarteritis nodosa, and deep mycoses. Serum lipase is a sensitive marker, and ghost cells are pathognomonic for PP.…”