[Cowden's disease. A report on two cases in Swiss families (author's transl)].
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.
D000328:Adult; D002648:Child; D005260:Female; D006222:Hamartoma / Q000235:genetics*; D006801:Humans; D007417:Intestinal Polyps / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009062:Mouth Neoplasms / Q000235:genetics* / Q000473:pathology; D009378:Neoplasms, Multiple Primary / Q000235:genetics* / Q000473:pathology; D010212:Papilloma / Q000235:genetics* / Q000473:pathology; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology; D013577:Syndrome; D014060:Tongue Diseases
Maladie de Cowden. Observation de deux familles suisses.