Pancreatic Panniculitis

Excerpt

First described by Hans Chiari in 1883, pancreatic panniculitis is a rare skin manifestation. Pancreatic panniculitis is a form of lobular panniculitis with fat necrosis within the panniculus and far from the pancreas. This occurs in chronic pancreatitis or pancreatic cancer patients, including acinar and islet cell carcinomas. Other pancreatic malignancies, including ductal adenocarcinoma, neuroendocrine tumors, and intraductal papillary mucinous neoplasms, have also been linked to this rare panniculitis.

The symptoms of pancreatic panniculitis include tender, erythematous to violaceous, ulcerated nodules that commonly appear on the upper and lower extremities. The nodules may undergo liquefaction and necrosis, leading to spontaneous ulceration and brown, viscous drainage. The condition is caused by the massive release of lipolytic enzymes into the bloodstream, which is attributed to the underlying pancreatic disease.

Patients with pancreatic panniculitis are often systemically ill with fever and weight loss. Joint manifestations may be prominent, and serum lipase and amylase levels are elevated. These signs and symptoms precede findings of a pancreatic etiology up to 49% of the time.

As there is a relationship between the unregulated release of pancreatic enzymes from pancreatic malignancies to the proposed pathophysiology of pancreatic panniculitis, this condition can also be considered a paraneoplastic syndrome.

Polyarthritis may present concurrently with pancreatic panniculitis, either proceeding or alongside pancreatic disease, which has been labeled a pancreatic disease, panniculitis, and polyarthritis (PPP) syndrome.