Kabir Kaiser F., Lotfollahzadeh Saran
Michigan State University, College of Osteopathic Medicine
Vascular Research, Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA, Department of Surgery, vascular and endovascular surgery section, Boston Medical Center, Boston, MA
First described by Hans Chiari in 1883, pancreatic panniculitis is a rare skin manifestation. Pancreatic panniculitis is a form of lobular panniculitis with fat necrosis within the panniculus and far from the pancreas. This occurs in chronic pancreatitis or pancreatic cancer patients, including acinar and islet cell carcinomas. Other pancreatic malignancies, including ductal adenocarcinoma, neuroendocrine tumors, and intraductal papillary mucinous neoplasms, have also been linked to this rare panniculitis. The symptoms of pancreatic panniculitis include tender, erythematous to violaceous, ulcerated nodules that commonly appear on the upper and lower extremities. The nodules may undergo liquefaction and necrosis, leading to spontaneous ulceration and brown, viscous drainage. The condition is caused by the massive release of lipolytic enzymes into the bloodstream, which is attributed to the underlying pancreatic disease. Patients with pancreatic panniculitis are often systemically ill with fever and weight loss. Joint manifestations may be prominent, and serum lipase and amylase levels are elevated. These signs and symptoms precede findings of a pancreatic etiology up to 49% of the time. As there is a relationship between the unregulated release of pancreatic enzymes from pancreatic malignancies to the proposed pathophysiology of pancreatic panniculitis, this condition can also be considered a paraneoplastic syndrome. Polyarthritis may present concurrently with pancreatic panniculitis, either proceeding or alongside pancreatic disease, which has been labeled a pancreatic disease, panniculitis, and polyarthritis (PPP) syndrome.
胰腺性脂膜炎于1883年由汉斯· Chiari首次描述，是一种罕见的皮肤表现。胰腺性脂膜炎是小叶性脂膜炎的一种形式，在脂膜内有脂肪坏死且远离胰腺。这发生在慢性胰腺炎或胰腺癌患者中，包括腺泡癌和胰岛细胞癌。其他胰腺恶性肿瘤，包括导管腺癌、神经内分泌肿瘤和导管内乳头状黏液性肿瘤，也与这种罕见的脂膜炎有关。胰腺性脂膜炎的症状包括压痛、从红斑到紫红色、溃疡的结节，通常出现在上肢和下肢。结节可能会发生液化和坏死，导致自发溃疡和棕色、黏稠的渗出物。这种情况是由于脂解酶大量释放到血液中所致，这归因于潜在的胰腺疾病。胰腺性脂膜炎患者通常全身不适，伴有发热和体重减轻。关节表现可能很突出，血清脂肪酶和淀粉酶水平升高。这些体征和症状在发现胰腺病因之前出现的时间高达49%。由于胰腺恶性肿瘤中胰腺酶的无节制释放与胰腺性脂膜炎的推测病理生理学之间存在关联，这种情况也可被视为一种副肿瘤综合征。多关节炎可能与胰腺性脂膜炎同时出现，要么先于胰腺疾病出现，要么与胰腺疾病同时出现，这种情况被称为胰腺疾病、脂膜炎和多关节炎（PPP）综合征。
