水疱を呈しアミロイドA蛋白の沈着を認めた全身性アミロイドーシス

・水疱を形成した反応性AAアミロイドーシスを経験した．

・水疱・血疱を伴ったアミロイドーシスに抗AA抗体陽性を伴った例は本邦，海外ともに少なく，今後の症例の集積が待たれる．

（「症例のポイント」より）

　

A case of AA amyloidosis with bullous formation

　

Miyake, Atsushi1）Hayashi, Yoshio1）Takimoto, Sonoko1）Takahashi, Koji1）Saito, Ikuo2）Ohmatsu, Hanako1） 1）Department of Dermatology, National Hospital Organization Sagamihara National Hospital 2）Department of Diagnostic Pathology, National Hospital Organization Sagamihara National Hospital

　

An 87-year-old female presented with hemorrhagic bullous skin lesions with purpura and scratch marks affecting the neck, chest, and axilla. She had a history of chronic heart failure, chronic renal failure, and dementia, but had not undergone dialysis. Biopsy specimen from purpura revealed amorphous, eosinophilic masses in the dermis and subcutaneous tissue. The direct fast scarlet stain revealed amyloid deposition. The amyloid was permanganate-sensitive, positive for anti-AA antibody, but negative for anti-TTR antibody; the observation qualified for a diagnosis of AA amyloidosis. She had no collagen diseases, including rheumatoid arthritis or systemic vasculitis. Bence-Jones protein was not detected. However, high titer of CA125, fecal occult blood, and IgG-λ type M-proteinemia were observed. Computed tomography showed thickened rectal wall. The evaluation ended, as she desired. There are only few reports of AA amyloidosis with blister formation in current literature.