monoclonal gammopathy of undetermined significance（MGUS）に伴う全身性アミロイドーシス

・自験例はAL（免グロブリン性）アミロイドーシスにみられる典型的な「アライグマの眼サイン（raccoon eyes sign）」を呈した．

・皮膚生検組織のCongo red染色においては，アミロイドの染色性が薄く，典型的な染色所見は得られなかったが，心筋生検組織にて明瞭なアミロイド沈着が証明され，病理組織学的にも全身性アミロイドーシスと確定診断した．

（「症例のポイント」より）

　

Systemic amyloidosis with monoclonal gammopathy of undetermined significance (MGUS)

　

Ueda, Takashi1）Utsumi, Yuri1）Watanabe, Masahiro2）Shirai, Kyomi1） 1）Department of Dermatology, National Hospital Organization Yokohama Medical Center 2）Department of Cardiology, National Hospital Organization Yokohama Medical Center

　

An 83-year-old Japanese man presented with an approximately 1-year history of purpura in the eyelids （raccoon eyes sign）. The purpura, which develops with minor trauma, gradually extended to the neck and trunk. Skin biopsy from an eyelid showed pink, acellular, eosinophilic, homogeneous material in the dermis on hematoxylin and eosin staining. This pink eosinophilic material displayed apple-green birefringence under polarized light on congo red staining. Bone marrow biopsy showed 10％ plasma cells. Systemic amyloidosis was diagnosed based on the clinical presentation and histopathology. Six months after first presentation, he fainted twice. A biopsy of cardiac muscle showed amyloid deposits resembling those from skin biopsy. The patient declined further treatment and died of heart failure after 1 year.