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Cowden's disease: A case report and literature review

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Abstract

Cowden's disease, or multiple hamartoma syndrome, is an uncommon condition with characteristic mucocutaneous lesions associated with abnormalities of the breast, thyroid, and gastrointestinal tract. We describe a 51-year-old man with hyperplastic polyposis of the entire alimentary tract as the most prominent feature of this disease. We also present a review of 85 cases of this entity as reported in the English medical literature, and summarize the pertinent findings.

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References

  1. Lloyd KM II, Dennis M: Cowden's disease. A possible new symptom complex with multiple system involvement.Ann Intern Med 58:136–142, 1963

    Google Scholar 

  2. Weary PE, Gorlin RJ, Gentry WC, Comer JE, Greer KE: Multiple hamartoma syndrome (Cowden's disease).Arch Dermatol 106:682–690, 1972

    Google Scholar 

  3. Siegel JM: Tuberous sclerosis (forme fruste) vs. Cowden syndrome.Arch Dermatol 110:476–477, 1974

    Google Scholar 

  4. Gentry WC, Eskritt NR, Gorlin RJ: Multiple hamartoma syndrome (Cowden disease).Arch Dermatol 109:521–525, 1974

    Google Scholar 

  5. Gentry WC, Reed WB, Siegel JM: Cowden disease.Birth Defects 11:137–141, 1975

    Google Scholar 

  6. Burnett JW, Goldner R, Calton GJ: Cowden disease: report of two additional cases.Br J Dermatol 93:329–336, 1975

    Google Scholar 

  7. Siegel JM: Cowden disease: report of a case with malignant melanoma.Cutis 16:255–258, 1975

    Google Scholar 

  8. Brownstein MH, Mehregan AH, Bikowski JB: Trichilemmomas in Cowden's disease.JAMA 238:26, 1977

    Google Scholar 

  9. Mulvihill JJ, McKeen EA: Discussion: genetics of multiple primary tumors. A clinical etiologic approach illustrated by three patients.Cancer 40:1867–1871, 1977

    Google Scholar 

  10. Nuss DD, Aeling JL, Clemons DE, Weber WN: Multiple hamartoma syndrome (Cowden's disease).Arch Dermatol 114:743–746, 1978

    Google Scholar 

  11. Wade TP, Kopf AW: Cowden's disease: a case report and review of the literature.J Dermatol Surg Oncol 4:459–464, 1978

    Google Scholar 

  12. Weinstock JV, Kawanishi H: Gastrointestinal polyposis with orocutaneous hamartomas (Cowden's disease).Gastroenterology 74:890–895, 1978

    Google Scholar 

  13. Brownstein MH, Wolf M, Bikowski JB: Cowden's disease. A cutaneous marker of breast cancer.Cancer 41:2393–2398, 1978

    Google Scholar 

  14. Emmerson RW: Epidermodysplasia verruciformis and punctate keratoderma.Br J Dermatol 103 (suppl 18):50–52, 1980

    Google Scholar 

  15. Gold BM, Bagla S, Zarrabi MH: Radiologic manifestations of Cowden disease.AJR 135:385–387, 1980

    Google Scholar 

  16. Gertzman GBR, Clark M, Gaston G: Multiple hamartoma and neoplasia syndrome (Cowden's syndrome).Oral Surg 49:314–316, 1980

    Google Scholar 

  17. Allen BS, Fitch MH, Smith JG Jr: Multiple hamartoma syndrome. A report of a new case with associated carcinoma of the uterine cervix and angioid streaks of the eyes.J Am Acad Dermatol 2:303–308, 1980

    Google Scholar 

  18. Hauser H, Ody B, Plojoux O, Wettstein P: Radiological findings in multiple hamartoma syndrome (Cowden disease). A report of three cases.Radiology 137:317–323, 1980

    Google Scholar 

  19. Yuasa T, Hanano M, Ohshima F, Tsubaki T: The association of myasthenia gravis with multiple hamartoma syndrome (Cowden disease).Ann Neurol 7:591–592, 1980

    Google Scholar 

  20. Ortonne JP, Lambert R, Daudet J, Berthet P, Gianadda E: Involvement of the digestive tract in Cowden's disease.Int J Dermatol 19:570–576, 1980

    Google Scholar 

  21. Bart RS, Kopf AW: Cowden's disease (multiple hamartoma syndrome).J Dermatol Surg Oncol 7:378–380, 1981

    Google Scholar 

  22. Elton R, Sroud J, Wagenberg H, Grekin J, Schwartz O: Cowden's syndrome.Int J Dermatol 20:617–618, 1981

    Google Scholar 

  23. Thyresson HN, Doyle JA: Cowden's disease (multiple hamartoma syndrome).Mayo Clin Proc 56:179–184, 1981

    Google Scholar 

  24. Ruschak PJ, Kauth YC, Luscombe HA: Cowden's disease associated with immunodeficiency.Arch Dermatol 117:573–575, 1981

    Google Scholar 

  25. Russell JR, O'Brien M, Wells RS: Cowden's syndrome.Br J Dermatol 105 (suppl 19):57–58, 1981

    Google Scholar 

  26. Aylesworth R, Vance JC: Multiple hamartoma syndrome with endometrial carcinoma and the sign of Leser-Trelat.Arch Dermatol 118:136–138, 1982

    Google Scholar 

  27. Sogol PB, Sugawara M, Gordon HE, Shellow WVR, Hernandez F, Hershman JM: Cowden's disease: familial goiter and skin hamartomas. A report of three cases.West J Med 139:324–328, 1983

    Google Scholar 

  28. Salem OS, Steck WD: Cowden's disease (multiple hamartoma and neoplasia syndrome). A case report and review of the English literature.J Am Acad Dermatol 8:686–696, 1983

    Google Scholar 

  29. Aram H, Zidenbaum M: Multiple hamartoma syndrome (Cowden's disease).J Am Acad Dermatol 9:774–776, 1983

    Google Scholar 

  30. Gorensek M, Matko I, Skralovnik A, Rode M, Satler J, Jutersek A: Disseminated hereditary gastrointestinal polyposis with orocutaneous hamartomatosis (Cowden's disease).Endoscopy 16:59–63, 1984

    Google Scholar 

  31. Starink TM: Cowden's disease: analysis of fourteen new cases.J Am Acad Dermatol 11:1127–1141, 1984

    Google Scholar 

  32. Carlson GJ, Nivatvongs S, Snover DC: Colorectal polyps in Cowden's disease (multiple hamartoma syndrome).Am J Surg Pathol 8:763–770, 1984

    Google Scholar 

  33. Gilbert HD, Plezia RA, Pietruk T: Cowden's disease (multiple hamartoma syndrome).J Oral Maxillofac Surg 43:457–460, 1985

    Google Scholar 

  34. Swart JGN, Lekkas C, Alleard RHB: Oral manifestations in Cowden's syndrome.Oral Surg Oral Med Oral Pathol 59:264–268, 1985

    Google Scholar 

  35. Halevy S, Sandbank M, Pick AI, Feuerman E: Cowden's disease in three siblings: electron-microscope and immunological studies.Acta Derm Venereol 65:126–131, 1985

    Google Scholar 

  36. Naylor EW, Lebenthal E: Gardner's syndrome: recent developments in research and management.Dig Dis Sci 25:945–959, 1980

    Google Scholar 

  37. Burt RW, Berenson MM, Lee RG, Tolman KG, Freston JW, Gardner EJ: Upper gastrointestinal polyps in Gardner's syndrome.Gastroenterology 86:295–301, 1984

    Google Scholar 

  38. Kilcheski T, Kressel HY, Laufer I, Rogers D: The radiographic appearances of the stomach in Cronkhite-Canada syndrome.Radiology 141:57–60, 1981

    Google Scholar 

  39. Johnson K, Soergel KH, Hensely GT, Dodds WJ, Hogan WJ: Cronkhite-Canada syndrome: gastrointestinal pathophysiology and morphology.Gastroenterology 63:140–152, 1972

    Google Scholar 

  40. Estrada R, Spjut HT: Hamartomatous polyps in Peutz-Jeghers syndrome, light-, histochemical, and electron-microscopic study.Am J Surg Pathol 7:747–754, 1983

    Google Scholar 

  41. Bulow S: Colorectal polyposis syndrome.Scand J Gastroenterol 19:289–293, 1984

    Google Scholar 

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Chen, Y.M., Ott, D.J., Wu, W.C. et al. Cowden's disease: A case report and literature review. Gastrointest Radiol 12, 325–329 (1987). https://doi.org/10.1007/BF01885173

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  • DOI: https://doi.org/10.1007/BF01885173

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