Pancreatic panniculitis M79.8

Chiari, 1883

Very rarely in the context of acute or chronic pancreatitis or lipase-forming tumours (adenocarcinomas of the pancreas), necroses of the subcutaneous fatty tissue occur. S.a. panniculitis, s.a. pancreatic diseases skin changes.

Rare disease. In 2-3% of all patients with pancreatic diseases.

The clinical symptoms are triggered by focal enzymatic effects of the lipases released by the pancreas (pancreatic lipase, phospholipase-A2) or alpha-amylase. Trypsin increases vascular permeability and allows enzymes to cross over into subcutaneous adipose tissue or bone marrow. Amylase and lipase cause fat breakdown, leading to saponification of neutral fat to glycerol and free fatty acids. Calcium soap is formed from these.

The release of fatty acids into the tissue causes an (aseptic) inflammatory tissue reaction.

This process is induced mainly in acute/chronic (alcohol-related) pancreatitis (2/3 of patients), less frequently in pancreatic carcinoma (1/3 of patients), after abdominal trauma or cholelithiasis, or as an early sign of liver carcinoma.

The panniculitic changes precede the diagnosis of the underlying disease in about half of the cases.

Mostly middle-aged men, often with an alcohol history.

Lower extremities (extensor and flexor sides), especially pretibial, more rarely trunk and buttocks. The lesions show a predilection for areas close to the joints.

Integument: Acute, often febrile clinical picture with episodic, painful, usually symmetrical, 5-7 cm large, markedly indurated, hyperthermic and reddened, subcutaneous, occasionally ulcerated nodules. The nodules may heal spontaneously leaving brown spots. Occasionally, sterile abscesses may develop from which a viscous, oily exudate is secreted. Consecutive ulcer formation is possible.

Extracutaneous manifestations: association with monoarthritis, polyarthritis, and/or polyserositis.

Arthritis can be very severe and lead to destruction of the joint. In about 10% of cases, aseptic bone necrosis is also detectable.

Abdominal symptoms may be associated with inflammatory involvement of the visceral adipose tissue (omentum) as well as the peritoneum.

The triad of panniculitis, pancreatitis, polyarthritis is also called PPP syndrome.

Overall, patients with complications such as arthritis or disseminated fat necrosis have a worse prognosis. PPP syndrome with high lipase levels are indications of a paraneoplastic etiology (Zundler S et al. 2016).

Increase of lipase and/or amylase, serum and urinary amylase, possibly gamma-GT and AP, leucocytosis, eosinophilia; hypocalcaemia. Increased inflammatory parameters (CRP; BSG).

Erythema nodosum (extensor sides of the lower legs)

Erythema induratum (dorsal side of the lower legs, especially in women)

Lupus panniculitis (upper arms, shoulder, face)

Panniculitis with alpha-1-antitrypsin deficiency

Panniculitic T-cell lymphomas

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