Yanagawa T, Yokoyama A, Noya K, Kasuga Y, Takei I, Maruyama H, Kataoka K, Saruta T, Kawamura S, Toyama K
Department of Internal Medicine, School of Medicine, Keio University, Tokyo, Japan.
South Med J. 1990 Nov;83(11):1323-6. doi: 10.1097/00007611-199011000-00026.
A 15-year-old boy had persistent fever with severe neutropenia, thrombocytopenia, coagulation disorder, and marked elevation of lactate dehydrogenase values. A diagnosis of cytophagic histiocytic panniculitis was made after repeated skin biopsies. Three years after onset, he gradually lost 20 kg in body weight, and both skin and bone marrow specimens revealed degenerative changes of fatty tissue without any inflammatory cells. These findings suggest that total lipodystrophy syndrome results from the inflammatory destructive process of adipose tissue. To our knowledge, our report is the first to show, in a series of histologic studies, that an inflammatory destructive process may be involved in some cases of total lipodystrophy syndrome.
一名15岁男孩持续发热，伴有严重中性粒细胞减少、血小板减少、凝血障碍以及乳酸脱氢酶值显著升高。经多次皮肤活检后诊断为噬血细胞性组织细胞性脂膜炎。发病三年后，他体重逐渐减轻了20千克，皮肤和骨髓标本均显示脂肪组织有退行性改变，未见任何炎症细胞。这些发现提示完全性脂肪营养不良综合征是由脂肪组织的炎症性破坏过程所致。据我们所知，在一系列组织学研究中，我们的报告首次表明炎症性破坏过程可能与某些完全性脂肪营养不良综合征病例有关。
