Cowden's Disease: A Possible New Symptom Complex with Multiple System Involvement

Abstract

Case Studies1 January 1963Cowden's DiseaseA Possible New Symptom Complex with Multiple System InvolvementKENNETH M. LLOYD II, M.D., MACEY DENNIS, M.D.KENNETH M. LLOYD II, M.D., MACEY DENNIS, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-58-1-136 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptIn recent years an accelerated interest has been directed toward a better understanding of the rare, familial, developmental diseases. The purpose of this communication is to define an unusual symptom complex which previously has not been described. Because of the multiplicity of findings, and the ill-defined classification for this disease, it shall be referred to as "Cowden's disease," the family name of the propositus. The clinical findings include: an adenoid facies; hypoplasia of the mandible and maxilla; a high-arch palate; hypoplasia of the soft palate and uvula; microstomia; papillomatosis of the lips and oral pharynx; scrotal tongue; multiple thyroid adenomas;...References1. MOEHLIGGODBOW RCJJ: The significance of the high-arched palate. Harper Hosp. Bull. 4: 168, 1946. MedlineGoogle Scholar2. SMITHSTOWE JLFR: The Piere Robin syndrome. Pediatrics 27: 128, 1961. Google Scholar3. SAGE RA: Some interesting lesions of the tongue. Arch. Otolaryng. 43: 122, 1946. CrossrefMedlineGoogle Scholar4. HALPERINKOLASJEFFERISHUDDLESTONROBINSON VSKRSOHG: The occurrence of Fordyce spots, benign migratory glossitis, median rhomboid glossitis and fissured tongue in 2,478 dental patients. Oral Surg. 6: 1072, 1953. CrossrefMedlineGoogle Scholar5. WALLKBLUEFARB SSM: Melkersson-Rosenthal syndrome. Arch. Derm. 84: 798, 1961. CrossrefMedlineGoogle Scholar6. FISHERRASHID AKPJ: Inflammatory papillary hyperplasia of the palatal mucosa. Oral Surg. 5: 191, 1952. CrossrefMedlineGoogle Scholar7. THOMA KH: Papillomatosis of the palate. Oral Surg. 5: 214, 1952. CrossrefMedlineGoogle Scholar8. CLARK DE: Association of irradiation with cancer of thyroid in children and adolescents. JAMA 159: 1007, 1955. CrossrefMedlineGoogle Scholar9. UHLMANN EM: Cancer of the thyroid and irradiation. JAMA 161: 504, 1956. CrossrefMedlineGoogle Scholar10. CONNALLY JE: Massive hypertrophy of the breasts. Stanford Med. Bull. 13: 393, 1955. MedlineGoogle Scholar11. GREIG DM: On pubertal mammary hypertrophy. Edinburgh Med. J. 18: 153, 1922. Google Scholar12. BYARSJURKIEWICZ LTM: Congenital macrogingivae and hypertrichosis with subsequent giant fibroadenomas of the breasts. Plast. Reconstr. Surg. 27: 608, 1961. CrossrefGoogle Scholar13. MCLAUGHLINSCHENKENTAMISIEA CWJRJX: A study of precancerous epithelial hyperplasia and noninvasive papillary carcinoma of the breasts. Ann. Surg. 153: 735, 1961. CrossrefMedlineGoogle Scholar14. SAINSBURY HK: Congenital funnel chest. Lancet 253: 615, 1947. CrossrefGoogle Scholar15. ELLIOTT JF: Polycystic disease of the liver. Canad. Med. Assoc. J. 66: 2, 1952. Google Scholar16. SIFREPHELPSCOLEKIMBALL RAHWNJCH: Non-parasitic cystic disease of the liver. Ann. Intern. Med. 42: 1288, 1955. LinkGoogle Scholar17. DAVIS CR: Non-parasitic cyst of the liver. Amer. J. Surg. 35: 590, 1937. CrossrefGoogle Scholar18. COMFORTGRAYDAKLINWHITESELL MWHKDCFB: Polycystic disease of the liver; a study of 24 cases. Gastroenterology 20: 60, 1952. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: KENNETH M. LLOYDII, M.D.; MACEY DENNIS, M.D.Affiliations: Youngstown, OhioFrom the Department of Medicine, Youngstown Hospital Association, Youngstown, Ohio.Requests for reprints should be addressed to Kenneth M. Lloyd, II, M.D., Henry Ford Hospital, Box 166, Detroit 2, Michigan. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byGASTROINTESTINAL CANCER SYNDROMESPhenolic Phytochemicals for Prevention and Treatment of Colorectal Cancer: A Critical Evaluation of In Vivo StudiesClinical Spectrum and Science Behind the Hamartomatous Polyposis SyndromesImpaired social cognition and fine dexterity in patients with Cowden syndrome associated with germline PTEN variantsA Case of Cowden Syndrome Presenting with Diverse Cutaneous ManifestationsPosterior Fossa Intra-Axial Lesion: There Are Pathologies Beyond MetastasesA progressive and refractory case of breast cancer with Cowden syndromeStrong Hereditary Predispositions to Colorectal CancerCowden Syndrome Case Report: Use of an Ultrasonic Surgical Aspirator for Cosmetic Removal of Lip HamartomasCowden Disease: A ReviewThyroid and renal cancers: A bidirectional associationA Rare Case of Cleft Palate Associated With Tongue Hamartoma: A Case Report and Systematic ReviewCase Report: Duodenal Carcinoma in a 40-Year-Old Asian Man With Cowden SyndromeImmature teratoma of the ovary associated with Cowden syndromeHormonal Crosstalk Between Thyroid and Breast CancerGastrointestina