Pancreatic Panniculitis - Dr Sampurna Roy MD

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.

Pancreatic panniculitis associated with acinar cell pancreatic carcinoma.J Cutan Med Surg. 2008 Jan-Feb;12(1):38-42.

BACKGROUND: Pancreatic panniculitis is a rare entity, occurring in less than 2% of patients with pancreatic disorders. Skin manifestations may precede the diagnosis of a pancreatic disease by many months. When treatable, correction of the underlying pancreatic disorder may lead to prompt resolution of the panniculitis.

OBJECTIVE: We present the case of a 74-year-old-man with a history of chronic pancreatitis who presented with an acute onset of tender, nonulcerating nodules. The clinical and histologic features of pancreatic panniculitis are discussed, with a brief review of the differential diagnosis and clinical approach to panniculitis.

CONCLUSIONS: Pancreatic panniculitis is a recognizable clinical entity with characteristic histologic features that may resolve with treatment of the underlying pancreatic disorder. The algorithm-based clinical approach to panniculitis presented in this study is a practical tool designed to guide clinicians in ordering investigations and determining the appropriate management for patients presenting with subcutaneous nodules.

Less frequent associations include pseudocyst, vasculopancreatic fistulas and pancreas divisum.

Pancreatic panniculitis manifests as painful or asymptomatic subcutaneous nodules or indurated plaques on the thighs, buttocks, lower trunk or distal parts of the lower extremities. Involvement of areas such as breasts, and abdomen are described.

There may also be polyserositis, arthritis, eosinophilia or rarely a leukemoid reaction.

Panniculitis has also been reported in association with an islet cell carcinoma.

Lesions probably result from the local action of blood-borne pancreatic lipase and trypsin, although other factors may also be involved.

Cases have been reported without pancreatic disease, but with circulating lipase or amylase of unknown origin.

Histopathology : Image1 ; Image2 ; Image3

Sections of established lesions show a lobular panniculitis involving much of the fat of the affected lobule. Image

Sometimes, contiguous lobules show a different stage in the histological evolution of the process. Image

Early lesions show enzymatic fat necrosis, with the ghost-like outline of fat cells remaining. Image

It has been suggested on the basis of one case, that at an even earlier stage (there is a septal panniculitis .

Liquefaction with breakdown of fat cells will eventually occur.

At the margins of the necrotic fat there is a variable neutrophil infiltrate, usually mild, associated with nuclear dusting, fine basophilic calcium deposits and some hemorrhage.

The necrotic fat cells may also have a pale basophilic hue due to the deposition of calcium salt.

In older lesions there are giant cells, lipophages, lymphocytes, hemosiderin and other blood pigment, and eventual fibrosis.

There may be some extension of the inflammatory process into the underlying dermis.

The treatment of pancreatic panniculitis is directed at the underlying pancreatic disease.

Usually those cases associated with pancreatitis slowly undergo resolution once the inflammatory episode of the pancreas regresses.

When there is involvement of subcutaneous fat other than the lower extremities, persistent disease, frequent relapses, or ulceration, the possibility of an occult underlying carcinoma of the pancreas should be considered.

Further reading:

Histopathology of panniculitis - aspects of biopsy techniques and difficulties in diagnosis.J Dtsch Dermatol Ges. 2011 Nov 16

Pancreatic panniculitis associated with acinar cell carcinoma of the pancreas: a case report.Ann Dermatol. 2011 May;23(2):225-8.

Panniculitis. Part II. Mostly lobular panniculitis.J Am Acad Dermatol. 2001 Sep;45(3):325-61

Pancreatic panniculitis: A rare form of panniculitis.Dermatology Online Journal 15 (3): 17

Pancreatic panniculitis.Dermatol Clin. 2008 Oct;26(4):465-70, vi.

Cutaneous and mucous mucormycosis mimicking pancreatic panniculitis and gouty panniculitis.J Am Acad Dermatol. 2011 Sep 27.

Pancreatic panniculitis: a cutaneous presentation as an initial clue to the diagnosis of pancreatic cancer.J Med Assoc Thai. 2011 Feb;94 Suppl 1:S253-7.