Clinical characteristics, treatment, and outcome of pancreatitis, panniculitis, and polyarthritis syndrome: a case-based review

Betrains, Albrecht; Rosseels, Wouter; Mieghem, Elke Van; Vanderschueren, Steven; Nijs, Jochen

doi:10.1007/s10067-020-05333-8

Cited by 17 publications

(23 citation statements)

References 51 publications

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“…middle-aged males with pancreatitis or pancreatic carcinoma, and lacks gastrointestinal symptoms in nearly half of cases. 1 However, it is extraordinarily associated with pancreatic neuroendocrine tumor (PanNET), with only three of 59 PPP syndrome cases and 14 pancreatic panniculitis (PP) cases caused by PanNET. 1,3 We report a middle-aged woman with PanNETinduced PPP syndrome.…”

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confidence: 99%

“…Empirical broad-spectrum antibiotics were prescribed for 10 days due to intermittent pyrexia (39.3°C) with negative blood The underlying mechanism of PPP syndrome could be involved in subcutaneous adiponecrosis and intramedullary lipolysis caused by pancreatic enzymes, especially lipase. 1,3 PP can precede, coincide, or succeed pancreatic conditions and often presents as tender, red-brown nodules on the lower limbs. 2 The nodules can ulcerate and release a brown lipid-rich material due to fat necrosis.…”

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confidence: 99%

“…2 The nodules can ulcerate and release a brown lipid-rich material due to fat necrosis. [1][2][3] The differential diagnosis includes erythema nodosum, erythema induratum, cutaneous polyarteritis nodosa, and deep mycoses. Serum lipase is a sensitive marker, and ghost cells are pathognomonic for PP.…”

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confidence: 99%

“…Serum lipase is a sensitive marker, and ghost cells are pathognomonic for PP. 1,3 Both hyperlipasemia and hyperamylasemia are seen in PP with acinar cell carcinoma and panniculitis, while hyperlipasemia and normoamylasemia are present in PP with PanNET. 3 Osteoarticular manifestations include mainly polyarthritis involving the ankles, knees, and wrists and occasionally polyserositis and intramedullary fat necrosis.…”

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confidence: 99%

“…3 Osteoarticular manifestations include mainly polyarthritis involving the ankles, knees, and wrists and occasionally polyserositis and intramedullary fat necrosis. 1,2,5 Intramedullary/ intraosseous fat necrosis presents as multifocal low-signal intensity lesions on T1-weighted imaging (T1WI) and high-signal intensity lesions on T2WI. 5 PanNETs originate from hormone-producing cells of the pancreas and are predominantly nonfunctioning.…”

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A case of pancreatitis‐panniculitis‐polyarthritis syndrome due to pancreatic neuroendocrine tumor

Song

Dai

et al. 2022

Int J Dermatology

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Approximately 80 million Americans have limited health literacy, and lower health literacy has been associated with both poorer access to care and health outcomes. 3 The American Medical Association and the National Health Institutes recommend that the readability of patient education materials be at reading grade level 6 or lower. 4 The average patient reads at grade level 7, and nearly 20% of Americans have difficulty comprehending reading grade level 4 text. [3][4][5] This indicates that currently available online resources for PV and BP are on average at least six reading grades higher than recommended and at least five grades higher than the average patient's reading ability. Remarkably, we found that resources written by medical doctors, and especially by dermatologists, had higher ARGL than those written by non-medical doctors.Limitations of our study included the cross-sectional design and possible multi-rater variability across the different validated readability measures. Additionally, using "pemphigus" and "pemphigoid" as search terms may have increased the grade level of resources. Our findings highlight the need to develop online patient education resources that are accessible to a higher proportion of patients and to actively involve content providers and patients in resource development.

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A case of pancreatitis‐panniculitis‐polyarthritis syndrome due to pancreatic neuroendocrine tumor

Song

Dai

et al. 2022

Int J Dermatology

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Panniculitis

Requena,

Cerroni

2024

Rook's Textbook of Dermatology

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The panniculitides represent a group of heterogeneous inflammatory diseases that involve the subcutaneous fat. The specific diagnosis of these disorders requires histopathological study because different panniculitides usually show monotonous clinical appearance, namely subcutaneous erythematous nodules on the lower extremities. Histopathological study of panniculitis is also challenging because of an inadequate clinicopathological correlation and the evolutionary nature of the lesions. Often, biopsy specimens are taken from late‐stage lesions, which results in non‐specific histopathological findings. In addition, large‐scalpel incisional biopsies are required. However, with adequate biopsy samples a histopathological differential diagnosis between a mostly septal and a mostly lobular panniculitis is straightforward and with adequate clinicopathological correlation, a specific diagnosis may be rendered in most cases of panniculitis. Mostly septal panniculitides with vasculitis include superficial thrombophlebitis and cutaneous polyarteritis nodosa (cutaneous arteritis). Septal panniculitides with no vasculitis may appear as the consequence of dermal inflammatory processes extending to the subcutaneous fat, such as necrobiosis lipoidica, scleroderma, subcutaneous granuloma annulare, rheumatoid nodule and necrobiotic xanthogranuloma, whereas in other cases the inflammatory process involves primarily the connective tissue septa of the subcutis with no participation of the overlying dermis. The most frequent septal panniculitis is erythema nodosum. In contrast, the most common lobular panniculitis with vasculitis is erythema induratum of Bazin (nodular vasculitis). Mostly lobular panniculitides without vasculitis comprise a large list of disorders, including sclerosing panniculitis (lipodermatosclerosis), subcutaneous fat necrosis of the newborn, panniculitis associated with connective tissue diseases, pancreatic panniculitis, 1 ‐antitrypsin deficiency‐associated panniculitis, infective panniculitis and factitious panniculitis. In recent years, several cases of both septal and lobular panniculitis have been described as a consequence of the administration of new drugs, including immune checkpoint inhibitors and BRAF inhibitors used as treatment of metastatic melanoma, tyrosine kinase and Bruton tyrosine kinase inhibitors used for treatment of leukemia and other haematological malignancies and tumour necrosis factor inhibitor drugs.

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Undifferentiated pleomorphic sarcoma of the pancreas with novel SARM1‐NTRK1 gene fusion and associated pancreatitis, panniculitis, and polyarthritis syndrome

Bou‐Maroun,

Hoff,

Joshi

et al. 2023

Pediatric Blood & Cancer

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LETTER TO THE EDITOR 3 of 3 F I G U R E 2 A, Schematic representation of the predicted SARM1-NTRK1 chimeric protein. ARM, Armadillo repeat domain; SAM, Sterile alpha motif; TIR, Toll/interleukin-1. B, Genomic structure depicting the location and orientation of the SARM1 and NTRK1 genes, and the retained exons in fusion transcripts receptor/resistance protein domain; SP, signal peptide; LRR, leucine-rich domain; IgC, immunoglobulin-like domain; TM, transmembrane domain; TK, tyrosine kinase domain.

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Clinical characteristics, treatment, and outcome of pancreatitis, panniculitis, and polyarthritis syndrome: a case-based review

Cited by 17 publications

References 51 publications

A case of pancreatitis‐panniculitis‐polyarthritis syndrome due to pancreatic neuroendocrine tumor

A case of pancreatitis‐panniculitis‐polyarthritis syndrome due to pancreatic neuroendocrine tumor

Panniculitis

Undifferentiated pleomorphic sarcoma of the pancreas with novel SARM1‐NTRK1 gene fusion and associated pancreatitis, panniculitis, and polyarthritis syndrome

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