Multifocal Fibrosclerosis with Hypertrophic Pachymeningitis and a Soft Tissue Mass around the Thoracic Vertebral Bodies: A Case Report with Review of the Literature

作者：Sato Mitsuto; Kodaira Minori; Ishii Wataru^*; Nakagawa Michitaka; Kishida Dai; Miyazaki Daigo; Shimojima Yasuhiro; Matsuda Masayuki; Uehara Takeshi; Ikeda Shu ichi

来源：Internal Medicine, 2015, 54(17): 2267-2272.

DOI：10.2169/internalmedicine.54.3230

• multifocal fibrosclerosis
• hypertrophic pachymeningitis
• a soft tissue mass around thoracic vertebral bodies
• IgG4-related disease
• granulomatosis with polyangiitis

Multifocal fibrosclerosis is the term used to represent a combination of similar fibrous lesions occurring at different anatomical sites. We herein report a hypertrophic pachymeningitis patient with a soft tissue mass around the thoracic vertebral bodies. A histopathological analysis of the biopsied tissues from both lesions showed dense fibrosis and a marked infiltration of lymphocytes and plasma cells, which lead to the diagnosis of multifocal fibrosclerosis. This pathological condition closely resembles that of IgG4-related disease and is a very rare combination of manifestations. Our case suggests that hypertrophic pachymeningitis patients need to also undergo a whole body examination.

• 出版日期2015