Brégeon C, Sentenac P, Queinnec J Y, Renier J C
Rev Rhum Mal Osteoartic. 1987 Feb;54(2):129-35.
If cutaneous panniculitis, made of dermo-hypodermic nodules, is the most constant manifestation of pancreatic cytosteatonecrosis, articular and bony localizations are quite frequent and sometimes the first manifestation of the disease. Articular lesions: monoarthritis, oligo-arthritis and mostly polyarthritis, often assume a very inflammatory picture; the synovial fluid is oily or puriform, and has a high content of polynuclear cells or macrophages with a foamy cytoplasm, lipid droplets, triglycerides, non-esterified fatty acids, and pancreatic enzymes; the most typical synovial lesion is a cytosteatonecrosis with adipocytes showing a huge lipidic vacuole. The bony lesions are mostly osteolytic, microgeodic lesions, or more extended ones, pseudo-tumoral without soft tissue invasion: more rarely, there is a periosteal thickening, bony infarctions, or epiphyseal osteonecrosis; these lesions do not always demonstrate a hyperfixation on scintigrams. The physiopathology of these various lesions is usually linked to a diffusion of pancreatic lipases, but other enzymes or enzymatic inhibitors also intervene, as well as a cytotoxic and local inflammatory activity of the fatty acids themselves. Surgical treatment of the pancreatic lesions, when possible, is the only treatment that is truly effective.
如果由真皮-皮下结节构成的皮肤脂膜炎是胰腺细胞脂肪坏死最常见的表现,那么关节和骨骼受累也相当常见,有时甚至是该病的首发表现。关节病变:单关节炎、少关节炎,多数为多关节炎,常呈现非常明显的炎症表现;滑液呈油性或脓性,多核细胞或具有泡沫状细胞质的巨噬细胞、脂滴、甘油三酯、非酯化脂肪酸及胰腺酶含量很高;最典型的滑膜病变是细胞脂肪坏死,脂肪细胞呈现巨大的脂质空泡。骨骼病变多数为溶骨性、微小地图状病变,或范围更广的、无软组织侵犯的假肿瘤样病变;更少见的是骨膜增厚、骨梗死或骨骺骨坏死;这些病变在骨闪烁图上并非总是表现为放射性浓聚。这些不同病变的病理生理学通常与胰腺脂肪酶的扩散有关,但其他酶或酶抑制剂也参与其中,脂肪酸本身的细胞毒性和局部炎症活性也有作用。胰腺病变若有可能,手术治疗是唯一真正有效的治疗方法。