Pancreatic panniculitis M79.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 12.05.2021

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Synonym(s)

enzymic panniculitis; Fatty tissue necrosis; Fatty tissue necrosis disseminated; Fatty tissue necrosis pancreatic; pancreatic fat necrosis; Pancreatic fat tissue necrosis; Pancreatic pancreatic paniculitis; Pancreatic panniculitis; Pancreatogenic panniculitis; Panniculitis pancreatic; Panniculitis pancreatogenic; PPP Syndrome

History
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Chiari, 1883

Definition
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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.

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

Etiopathogenesis
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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.

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

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

Clinical features
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Integument: Acute, often febrile clinical picture with episodic, painful, usually symmetrical, 5-7 cm large, clearly indurated, overheated 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 ulceration is possible.

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

Arthritis may be very severe and lead to destruction of the joint. In10% 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.

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

Histology
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Lobular fat tissue necrosis with basophilic calcium deposits and nuclear fat cells. Mixed inflammatory infiltrate. Destructed adipocytes are abscess-like surrounded by dense infiltrates of neutrophil granulocytes, with foam cells and micropseudocytes in between.

Differential diagnosis
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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

Therapy
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Treatment of the underlying disease by internists. External treatment with antiphlogistic ointments or gels such as indomethacin (e.g. Amuno gel) or ibuprofen (e.g. Dolgit cream).

Literature
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  1. Arbeláez-Cortés A et al (2014) Polyarthritis and pancreatic panniculitis associated with pancreatic carcinoma: review of the literature. J Clin Rheumatol 20:433-436.
  2. Arenbergerova M et al. (2015) Pancreatic panniculitis associated with multiple osteolytic foci. dermatologist 66: 114-116
  3. Belgium AS, Sowden J (2004) Widespread panniculitis secondary to occult metastatic pancreatic lipase-secreting acinar cell carcinoma. Br J Dermatol 151 (Suppl 68): 32-33
  4. Braun-Falco O et al (1989) Pancreatogenic panniculitis. Dermatologist 40: 778-781
  5. Chiari H (1883) About the so-called fat necrosis. Prague Med Weekly 8: 285-286
  6. Corazza M et al (2003) Pancreatic panniculitis as a first sign of liver carcinoma. Acta Derm Venereol 83: 230-231
  7. Diaz-Cascajo C, Borghi S (2002) Subcutaneous pseudomembranous fat necrosis: new observations. J Cutan catholic 29: 5-10
  8. Hughes PSH et al (1975) Subcutaneous fat necrosis associated with pancreatic disease. Arch Dermatol 111: 506-510
  9. Kolb-Mäurer A (2015) Panniculitis in pancreatitis.JDDG 13: 807-809
  10. Krahl D (1991) Pancreatogenic panniculitis of indirect paraneoplastic origin. Act Dermatol 17: 281-283
  11. Requena L, Sanchez Yus E (2001) Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 45: 325-361

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 12.05.2021