A Case of Cowden Disease with Triple Extramammary Paget’s Disease

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  • triple Paget病を伴ったCowden病の1例
  • triple Pagetビョウ オ トモナッタ Cowdenビョウ ノ 1レイ

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Abstract

We present a case of Cowden disease with triple extramammary Paget’s disease. An 81-year-old man with red-colored plaques on the bisaxillary region and scrotum was referred to our clinic. Histological examination showed Paget’s cells in the epidermis. We diagnosed triple extramammary Paget’s disease. The patient also had multiple papules on the buccal mucosa and multiple keratotic papules on both hands. Gastrofiberoscopy and colonoscopy revealed multiple gastrointestinal polyps. Computed tomography images revealed a thyroid tumor. This patient was positive for a mutation in the PTEN gene, which resulted in Cowden disease. This syndrome is a systemic disorder involving hamartomatous overgrowth of tissues of all three embryonic origins and is associated with increased risks for thyroid, breast, and possibly other cancers. To our knowledge, this is a first case report of Cowden disease with extramammary Paget’s disease.

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