Aronson I K, West D P, Variakojis D, Malkinson F D, Wilson H D, Zeitz H J
J Am Acad Dermatol. 1985 Mar;12(3):535-51. doi: 10.1016/s0190-9622(85)70076-x.
The Weber-Christian syndrome (relapsing nodular panniculitis) displays a clinical spectrum varying from short, self-limited, or intermittent disease episodes to persistent disease with fatal outcome. Inflamed adipose tissue is exclusively subcutaneous in some patients and is both subcutaneous and perivisceral in others. Inflammation of fat may induce a focal cutaneous or a systemic extracutaneous histiocytic proliferative response in which hemophagocytosis may be a frequent characteristic. Major causes of death--sepsis, hepatic failure, hemorrhage, and thrombosis--are identical in the patients with and without the systemic histiocytic proliferation. Inflammation in fat, of and by itself, may be associated with significant morbidity and mortality, regardless of specific histopathology or inciting factors.
韦贝尔-克里斯琴综合征(复发性结节性脂膜炎)的临床谱各异,从短暂的、自限性的或间歇性发作到持续存在并导致致命后果的疾病。在一些患者中,炎症性脂肪组织仅位于皮下,而在另一些患者中则同时累及皮下和内脏周围。脂肪炎症可能引发局灶性皮肤或全身性皮肤外组织细胞增殖反应,其中噬血细胞现象可能是常见特征。无论有无全身性组织细胞增殖,主要死亡原因——败血症、肝衰竭、出血和血栓形成——在患者中是相同的。脂肪炎症本身可能与显著的发病率和死亡率相关,而与特定的组织病理学或诱发因素无关。