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April 1974

Multiple Hamartoma Syndrome (Cowden Disease)

Arch Dermatol. 1974;109(4):521-525. doi:10.1001/archderm.1974.01630040031006

Seven members in three generations of a family have multiple hamartoma syndrome (Cowden disease). Skin changes, characterized by progressive verrucous, papular, and lichenoid lesions involving mucosal and cutaneous surfaces, serve as hallmarks of associated fibrocystic breast disease, carcinoma of the breast, thyroid tumors, gastrointestinal polyposis, nervous system abnormalities, fibromas, angiomas, and lipomas. The syndrome is inherited in an autosomal dominant pattern. It is important to recognize the widespread and diverse mucocutaneous manifestations as signs of possible anatomic, metabolic, and internal abnormalities.