HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
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Occurrence/EpidemiologyThis section has been translated automatically.
Rare disease. In 2-3% of all patients with pancreatic diseases.
EtiopathogenesisThis section has been translated automatically.
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.
ManifestationThis section has been translated automatically.
Mostly middle-aged men, often with an alcohol history.
LocalizationThis section has been translated automatically.
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 featuresThis section has been translated automatically.
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.
LaboratoryThis section has been translated automatically.
Increase of lipase and/or amylase, serum and urinary amylase, possibly gamma-GT and AP, leucocytosis, eosinophilia; hypocalcaemia. Increased inflammatory parameters (CRP; BSG).
HistologyThis section has been translated automatically.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Arbeláez-Cortés A et al (2014) Polyarthritis and pancreatic panniculitis associated with pancreatic carcinoma: review of the literature. J Clin Rheumatol 20:433-436.
- Arenbergerova M et al. (2015) Pancreatic panniculitis associated with multiple osteolytic foci. dermatologist 66: 114-116
- 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
- Braun-Falco O et al (1989) Pancreatogenic panniculitis. Dermatologist 40: 778-781
- Chiari H (1883) About the so-called fat necrosis. Prague Med Weekly 8: 285-286
- Corazza M et al (2003) Pancreatic panniculitis as a first sign of liver carcinoma. Acta Derm Venereol 83: 230-231
- Diaz-Cascajo C, Borghi S (2002) Subcutaneous pseudomembranous fat necrosis: new observations. J Cutan catholic 29: 5-10
- Hughes PSH et al (1975) Subcutaneous fat necrosis associated with pancreatic disease. Arch Dermatol 111: 506-510
- Kolb-Mäurer A (2015) Panniculitis in pancreatitis.JDDG 13: 807-809
- Krahl D (1991) Pancreatogenic panniculitis of indirect paraneoplastic origin. Act Dermatol 17: 281-283
- Requena L, Sanchez Yus E (2001) Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 45: 325-361
Incoming links (10)Alcohol skin changes; Eosinophilia skin changes; Erythema nodosum; Fat tissue necrosis, pancreatic; Fatty tissue necrosis, disseminated; Lipoatrophy, localized after glucocorticosteroid injections; Pancreatic carcinoma; Pancreatic diseases skin changes; Panniculitis nodularis nonsuppurativa; Ppp syndrome;
Outgoing links (6)Acute pancreatitis; Erythema nodosum; Nodular vasculitis; Pancreatic carcinoma; Pancreatic diseases skin changes; Panniculitis (overview);
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