[Cowden's disease. A report on two cases in Swiss families (author's transl)]. 1979

R Kuffer, and M Rougier, and P Laugier, and G Fiore-Donno

Described by Lloyd and Dennis in 1963, Cowden's disease is a familial affection in which are found associated lesions of the buccopharyngeal mucosa (papillomatosis, papules, scrotal tongue), various types of skin lesions (verruciform keratotic papules, papilloma, lipoma, angioma, etc...), visceral manifestations usually related to lesions in the thyroid, breasts, or digestive tract (hamartoma or malignant tumors), and skeletal dysmorphism. The two patients were from different families, were aged 65 and 28 years, and had presented for consultation because of buccal mucous membrane papillomatosis, and in one case, hemorrhagic gingivitis. Cowden's disease was diagnosed from the clinical examination, past history, and investigations after hospital admission. The main value of the mucosal and cutaneous lesions is their diagnostic significance, enabling a synthesis of the multiple dispersed manifestations to be made, and a possible visceral tumor to be discovered in the early stages.

UI MeSH Term Description Entries
D007417 Intestinal Polyps Discrete abnormal tissue masses that protrude into the lumen of the INTESTINE. A polyp is attached to the intestinal wall either by a stalk, pedunculus, or by a broad base. Intestinal Polyp,Polyp, Intestinal,Polyps, Intestinal
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009062 Mouth Neoplasms Tumors or cancer of the MOUTH. Cancer of Mouth,Mouth Cancer,Oral Cancer,Oral Neoplasms,Cancer of the Mouth,Neoplasms, Mouth,Neoplasms, Oral,Cancer, Mouth,Cancer, Oral,Cancers, Mouth,Cancers, Oral,Mouth Cancers,Mouth Neoplasm,Neoplasm, Mouth,Neoplasm, Oral,Oral Cancers,Oral Neoplasm
D009378 Neoplasms, Multiple Primary Two or more abnormal growths of tissue occurring simultaneously and presumed to be of separate origin. The neoplasms may be histologically the same or different, and may be found in the same or different sites. Neoplasms, Synchronous,Neoplasms, Synchronous Multiple Primary,Multiple Primary Neoplasms,Multiple Primary Neoplasms, Synchronous,Synchronous Multiple Primary Neoplasms,Synchronous Neoplasms,Multiple Primary Neoplasm,Neoplasm, Multiple Primary,Neoplasm, Synchronous,Primary Neoplasm, Multiple,Primary Neoplasms, Multiple,Synchronous Neoplasm
D010212 Papilloma A circumscribed benign epithelial tumor projecting from the surrounding surface; more precisely, a benign epithelial neoplasm consisting of villous or arborescent outgrowths of fibrovascular stroma covered by neoplastic cells. (Stedman, 25th ed) Papilloma, Squamous Cell,Papillomatosis,Papillomas,Papillomas, Squamous Cell,Papillomatoses,Squamous Cell Papilloma,Squamous Cell Papillomas
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005260 Female Females
D006222 Hamartoma A focal malformation resembling a neoplasm, composed of an overgrowth of mature cells and tissues that normally occur in the affected area. Hamartomas
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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