Berkovic D, Hallermann C
Department of Internal Medicine, University Clinic Göttingen, Germany.
Onkologie. 2003 Oct;26(5):473-6. doi: 10.1159/000072982.
On rare occasions tumours of the pancreas produce high amounts of pancreatic lipase. The enzyme activity in the blood and in different tissues causes a syndrome called nodular panniculitis by focal necrosis of lipids and a concomittant inflammatory reaction.
A 72-year-old man was admitted to the dermatology clinic with the diagnosis of erythema nodosum. The patient had been well until 3 months earlier when painful red nodes developed on the skin of both shanks. He complained of profuse night sweating and a weight loss of 10 kg within that time but did not have fever. He also had noticed a painful swelling of his right index finger, left middle finger and the third toe on his left foot. Biopsy of the nodes revealed a focal necrosis of fatty tissue. Laboratory examinations showed a highly elevated concentration of serum pancreatic lipase. Further investigations showed a tumour in the pancreas and several osteolytic lesions. Tumour biopsy revealed a neuroendocrine carcinoma. After tumour resection serum lipase level immediately fell to almost normal values, and all skin and bone manifestations disappeared quickly.
Due to its clinical appearance the panniculitis syndrome is most often mistaken for either erythema nodosum or rheumatoid arthritis. A resection of the tumour after correct diagnosis should always be considered because the widespread manifestations in the skin and bones do not represent distant metastasis and have a very good chance to dissolve completely.
胰腺肿瘤在极少数情况下会产生大量胰脂肪酶。血液和不同组织中的酶活性通过脂质的局灶性坏死和伴随的炎症反应导致一种称为结节性脂膜炎的综合征。
一名72岁男性因结节性红斑诊断入住皮肤科诊所。患者此前一直健康,直到3个月前双侧小腿皮肤出现疼痛性红色结节。他抱怨在此期间盗汗严重且体重减轻了10公斤,但无发热。他还注意到右手食指、左手中指和左脚第三趾疼痛性肿胀。结节活检显示脂肪组织局灶性坏死。实验室检查显示血清胰脂肪酶浓度显著升高。进一步检查发现胰腺有肿瘤及多处溶骨性病变。肿瘤活检显示为神经内分泌癌。肿瘤切除后血清脂肪酶水平立即降至几乎正常,所有皮肤和骨骼表现迅速消失。
由于其临床表现,脂膜炎综合征最常被误诊为结节性红斑或类风湿关节炎。正确诊断后应始终考虑切除肿瘤,因为皮肤和骨骼的广泛表现并非远处转移,且有很大机会完全消退。