[Cowden's disease. Syndrome of multiple hamartomas].
Cowden's disease is a phacomatosis (multiple hamartomas), which is a familial, hereditary, dominant autosomal affection presenting as typical buccolabial mucocutaneous lesions associated with digestive tract polyps. Differential diagnosis of the predominantly digestive form of the disease is polyposis. A new case of this rare disorder is reported, only 62 cases being documented in literature.
D000293:Adolescent; D003111:Colonic Polyps / Q000235:genetics; D004067:Digestive System Neoplasms / Q000235:genetics; D006222:Hamartoma / Q000235:genetics*; D006801:Humans; D007414:Intestinal Neoplasms / Q000235:genetics; D008046:Lip / Q000473:pathology; D008297:Male; D009061:Mouth Mucosa / Q000473:pathology; D009062:Mouth Neoplasms / Q000235:genetics; D009378:Neoplasms, Multiple Primary / Q000235:genetics*; D012878:Skin Neoplasms / Q000235:genetics; D013577:Syndrome
La maladie de Cowden. Syndrome des hamartomes multiples.