Pancreatic disease, panniculitis, polyarthrtitis syndrome successfully treated with total pancreatectomy: Case report and literature review

Ferri, Valentina; Ielpo, Benedetto; Durán, Hipólito; Dı́az, Eduardo; Fabra, Isabel; Caruso, Riccardo; Malavé, L.; Plaza, Carlos; Rodríguez, Silvia; Garcia, Lina; Pérez, Virginia; Quijano, Yolanda; Vicente, Emilio

doi:10.1016/j.ijscr.2016.09.019

Cited by 28 publications

(30 citation statements)

References 17 publications

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“…Since this report, there have been few documentations of the condition and this may be due to misleading signs and symptoms that lead diagnosis of the condition and therefore incidence could be greatly under-reported. A review of the published cases reveals that, similarly to the patient we have reported, the majority of patients do not present with abdominal pain,5 and up to 40% of patients may present with panniculitis or arthritis 6. This may be due to a large number of cases of PPP syndrome being secondary to malignancy.…”

Section: Discussionsupporting

confidence: 64%

Unusual presentation of pancreatitis with extrapancreatic manifestations

2018

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Pancreatitis, panniculitis and polyarthritis syndrome (PPP syndrome) is a rare and complex manifestation of pancreatitis that is currently reported in few cases. We present this case regarding a 77-year-old man who presented unusually with a 6-week history of bilateral lesions on his lower limbs. During this time, he experienced increasing fatigue, joint pain, and reported fevers and significant weight loss. An incidental serum amylase was found to be significantly elevated and remained elevated throughout his 2-month hospital stay. He never complained of abdominal pain or other typical symptoms of pancreatitis. Histological examination of the leg lesions reported evidence of fat necrosis and changes consistent with pancreatic panniculitis. An abdominal CT scan revealed a well-defined lesion posterior to the neck of the pancreas. Further examination by endoscopic ultrasound revealed chronic inflammatory changes. The patient experienced a long admission and was managed supportively until he was well enough to be discharged home.

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Section: Discussionsupporting

confidence: 64%

Unusual presentation of pancreatitis with extrapancreatic manifestations

2018

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“…In the case of pancreatic cancer, surgery was recommended and endoscopic procedures such as pancreatic stenting have also been described. Steroids, non-steroidal anti-inflammatory drugs and immunosuppressants for skin lesions or arthritis have been found to be ineffective [15]. In the case presented in this report, the patient refused the mass resection and chemotherapy for treatment of pancreatic NET.…”

Section: Discussionmentioning

confidence: 81%

Pancreatic Neuroendocrine Tumor Presenting with Arthritis and Panniculitis

et al. 2017

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Pancreatic neoplasm is complicated and can be preceded by extra-pancreatic manifestations, such as cutaneous and musculoskeletal symptoms. Awareness of these associations is important for timely diagnosis and appropriate treatment. We report a case of pancreatic neuroendocrine tumor (NET) presenting with arthritis and panniculitis. The patient had a two month history of right knee pain and subcutaneous nodules in both legs. Synovial fluid analysis from the right knee joint revealed a mildly increased white blood cell count without crystallization. A skin biopsy of a subcutaneous nodule revealed lobular panniculitis. The initial treatment with empirical antibiotics did not alleviate the symptoms; however, the right knee arthritis and skin nodules improved with steroid treatment. On the eighth day of hospitalization, the patient complained of abdominal discomfort. Abdominopelvic computed tomography scanning revealed a 14-cm sized pancreatic mass with peritoneal metastasis. Percutaneous needle biopsy confirmed the diagnosis of pancreatic NET. (J Rheum Dis 2017;24:313-317)

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“…Thus DISCUSSION pancreatic pathology is typically the last of the triad to be detected. 8 The delay in diagnosing the etiology of the debilitating arthralgias in our patient is due to PPP syndromes' rarity and his disordered presentation. To our knowledge, our patient is the first case in which cutaneous manifestations appeared months later than the other components of the syndrome.…”

mentioning

confidence: 75%

“…6 Management of PPP syndrome is frequently aimed at treating the underlying pancreatic disease. 8 Panniculitis typically resolves following the resolution of the acute inflammation of the pancreatic parenchyma. Thus, supportive treatment, such as fluids and pain control, is all that is required.…”

mentioning

confidence: 99%

Disordered Presentation of Paraneoplastic Pancreatitis, Polyarthritis and Panniculitis (PPP) Syndrome in a Patient with End-Stage Pancreatic Cancer

Maarouf¹,

Wickenheiser²,

Sligh³

et al. 2018

J of Skin

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We report a rare case of PPP due to stage IV beta-islet pancreatic cancer presenting with polyarthritis, whose diagnosis was delayed due to an incomplete presentation of PPP syndrome. Following an extensive one-month infectious disease workup, tender erythematous papules began to develop, which prompted the consideration and subsequent biopsy for pancreatic panniculitis. Thus, the diagnosis was delayed due to a lagging cutaneous presentation—a component of the triad that is commonly the initial presentation in most patients.

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Pancreatic disease, panniculitis, polyarthrtitis syndrome successfully treated with total pancreatectomy: Case report and literature review

Cited by 28 publications

References 17 publications

Unusual presentation of pancreatitis with extrapancreatic manifestations

Unusual presentation of pancreatitis with extrapancreatic manifestations

Pancreatic Neuroendocrine Tumor Presenting with Arthritis and Panniculitis

Disordered Presentation of Paraneoplastic Pancreatitis, Polyarthritis and Panniculitis (PPP) Syndrome in a Patient with End-Stage Pancreatic Cancer

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