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European Journal of Dermatology

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Extramammary Paget's disease occurring in the context of Cowden syndrome: true association or mere coincidence? Volume 25, issue 1, January-February 2015

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Authors
1 Department of Dermatology,
2 Department of Pathology,
Graduate School of Medicine,
University of Toyama,
2630 Sugitani,
Toyama 930-0194,
Japan
3 Department of Dermatology,
Shinseikai Toyama Hospital,
Imizu,
Japan
4 Department of Dermatology,
Toyama Prefectural Central Hospital,
Toyama,
Japan

Cowden syndrome (CS) is a rare inherited disorder characterized by multiple hamartomas and an increased risk of carcinomas, such as thyroid and breast cancers [1]. Characteristic mucocutaneous manifestations include multiple facial trichilemmomas, oral mucosal papillomatoses and acral keratosis. A mutation in the phosphatase and tensin homolog (PTEN) gene has been identified as the cause of CS [2]. The present report describes a case of extramammary Paget's disease (EMPD) which developed in a Japanese [...]