“… 58 [ 89 ] 7 m acute pancreatitis resolution 59 [ 14 ] 61 m metastatic NEC of unknown primary site death a few weeks after first skin lesion 60 [ 42 ] 50 f metastatic ACC regression of skin lesions and tumor under octreotide, gemcitabine, streptozocin and doxorubicin; return after discontinuation; response to restart of therapy; follow-up 9 months after first skin lesion 61 [ 90 ] 74 m pancreatic ACC death 3.75 months after first skin lesion 62 [ 91 ] 61 m metastatic ACC n.r. 63 [ 92 ] 79 m metastatic pancreatic NEC Regression under cefazolin, dexamethasone and NSAID; death 13 months after first skin lesion 64 [ 93 ] 50 m acute pancreatitis death 38 days after first skin lesion 65 [ 94 ] 4 m acute pancreatitis resolution 66 [ 11 ] 45 m acute pancreatitis Regression under NSAID and prednisone 67 [ 95 ] 72 m acute pancreatitis resolution 68 [ 96 ] 52 f pancreatic carcinoma death six months after first skin lesion 69 ...…”
BackgroundPancreatic panniculitis is a rare condition, which has only been described in relation with pancreatic diseases up to now. It is characterized by necrotizing subcutaneous inflammation and is thought to be triggered by adipocyte necrosis due to systemic release of pancreatic enzymes with consecutive infiltration of neutrophils. We present the first case of a patient with pancreatic panniculitis caused by pancreatic-type primary acinar cell carcinoma (ACC) of the liver and without underlying pancreatic disease.Case presentationA 73-year old Caucasian female patient was referred to our department with painful cutaneous nodules persisting for eight weeks and with marked lipasemia (~15000 U/l; normal range <60 U/l). Four weeks prior, several liver lesions had been detected. Empiric treatment with steroids did not show any effect. A biopsy of the skin nodules revealed “pancreatic” panniculitis, while abdominal imaging with ultrasound, computed tomography and magnetic resonance imaging detected no abnormal pancreatic findings. Ultrasound-guided biopsy of the liver lesions showed infiltrates of an ACC. The patient died soon thereafter. Autopsy failed to reveal any other primary for the ACC, so that a pancreatic-type ACC of the liver was diagnosed by exclusion.One hundred thirty cases of pancreatic panniculitis published within the last 20 years are reviewed. ACC of the pancreas is the most common underlying neoplastic condition. Patients with associated neoplasm are significantly older, take longer to be diagnosed and have higher lipase levels than patients with underlying pancreatitis.Extrapancreatic pancreatic-type ACC is very rare, but shows the same biological features as ACC of the pancreas. It is believed to develop from metaplastic or ectopic pancreatic tissue. Up to now, no pancreatic panniculitis in extrapancreatic ACC has been described.ConclusionPancreatic panniculitis should always be included in the differential diagnosis of lipolytic panniculitic lesions. It can be regarded as a facultative paraneoplastic phenomenon.When suspected, a thorough work-up for identification of the underlying disease is mandatory and extrapancreatic lesions (e.g. liver) should also be considered. While administration of octreotide or steroids can sometimes alleviate symptoms, immediate treatment of the associated condition is the only effective management option.
“… 58 [ 89 ] 7 m acute pancreatitis resolution 59 [ 14 ] 61 m metastatic NEC of unknown primary site death a few weeks after first skin lesion 60 [ 42 ] 50 f metastatic ACC regression of skin lesions and tumor under octreotide, gemcitabine, streptozocin and doxorubicin; return after discontinuation; response to restart of therapy; follow-up 9 months after first skin lesion 61 [ 90 ] 74 m pancreatic ACC death 3.75 months after first skin lesion 62 [ 91 ] 61 m metastatic ACC n.r. 63 [ 92 ] 79 m metastatic pancreatic NEC Regression under cefazolin, dexamethasone and NSAID; death 13 months after first skin lesion 64 [ 93 ] 50 m acute pancreatitis death 38 days after first skin lesion 65 [ 94 ] 4 m acute pancreatitis resolution 66 [ 11 ] 45 m acute pancreatitis Regression under NSAID and prednisone 67 [ 95 ] 72 m acute pancreatitis resolution 68 [ 96 ] 52 f pancreatic carcinoma death six months after first skin lesion 69 ...…”
BackgroundPancreatic panniculitis is a rare condition, which has only been described in relation with pancreatic diseases up to now. It is characterized by necrotizing subcutaneous inflammation and is thought to be triggered by adipocyte necrosis due to systemic release of pancreatic enzymes with consecutive infiltration of neutrophils. We present the first case of a patient with pancreatic panniculitis caused by pancreatic-type primary acinar cell carcinoma (ACC) of the liver and without underlying pancreatic disease.Case presentationA 73-year old Caucasian female patient was referred to our department with painful cutaneous nodules persisting for eight weeks and with marked lipasemia (~15000 U/l; normal range <60 U/l). Four weeks prior, several liver lesions had been detected. Empiric treatment with steroids did not show any effect. A biopsy of the skin nodules revealed “pancreatic” panniculitis, while abdominal imaging with ultrasound, computed tomography and magnetic resonance imaging detected no abnormal pancreatic findings. Ultrasound-guided biopsy of the liver lesions showed infiltrates of an ACC. The patient died soon thereafter. Autopsy failed to reveal any other primary for the ACC, so that a pancreatic-type ACC of the liver was diagnosed by exclusion.One hundred thirty cases of pancreatic panniculitis published within the last 20 years are reviewed. ACC of the pancreas is the most common underlying neoplastic condition. Patients with associated neoplasm are significantly older, take longer to be diagnosed and have higher lipase levels than patients with underlying pancreatitis.Extrapancreatic pancreatic-type ACC is very rare, but shows the same biological features as ACC of the pancreas. It is believed to develop from metaplastic or ectopic pancreatic tissue. Up to now, no pancreatic panniculitis in extrapancreatic ACC has been described.ConclusionPancreatic panniculitis should always be included in the differential diagnosis of lipolytic panniculitic lesions. It can be regarded as a facultative paraneoplastic phenomenon.When suspected, a thorough work-up for identification of the underlying disease is mandatory and extrapancreatic lesions (e.g. liver) should also be considered. While administration of octreotide or steroids can sometimes alleviate symptoms, immediate treatment of the associated condition is the only effective management option.
“…The probable mechanism is that lipase and amylase from damaged pancreas tissue enters to circulation and affects subcutaneous fat lobules. [9] Pancreatic panniculitis typically presents as tender, edematous, and erythematous to red-brown subcutaneous nodules, most commonly involves lower legs, arms, and abdomen. [10]…”
Clinicians should be aware of the manifestation of multiple pancreatic pseudocyst and pancreatic panniculitis, and endoscopic transpapillary drainage may be a effective way in this scenario.
“…14 ). Clinically, panniculitis is manifested by erythematous and painful subcutaneous nodules that may ulcerate, with brown-colored, viscous to oily fluid exiting the ulcer as a sign of colliquative fat necrosis 15 . Panniculitis is most commonly localized in the distal parts of the lower limbs (Fig.…”
Although symptoms of pancreatic diseases such as pancreatitis, acute and chronic and, carcinoma of the pancreas are mainly gastrointestinal in nature, the extra-pancreatic symptoms are also important. These include skin symptoms, such as pancreatic panniculitis, acanthosis nigricans, livedo reticularis, necrolytic migratory erythema, cutaneous signs of hemorrhage, as in persons with severe acute pancreatitis, or the finding of cutaneous metastases of pancreatic carcinoma, which may be a sign of advanced disease. The pancreas is therefore one of those organs for which diagnosis and therapy are often multidisciplinary. In this review article, we summarize current knowledge of the possible skin manifestations of pancreatic disorders.
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