Abstract For the past eight years we have had under our care a remarkable treatment-resistant progressive case of Darier's disease. We should like at this time to summarize certain findings in this patient. Despite the fact that many of these studies are largely of a negative type, they are presented in the hope that they will stimulate further research in this challenging and baffling disease. Report of a Case Case 1.—This patient, now 18 years old, was first seen as a boy of 10 years. A scaling pruritic plaque had first appeared on his scalp at the age of 9 years. This rapidly extended over the entire scalp and on to the forehead. At the time of his initial visit he presented the classic keratotic lesion of Darier's disease (Fig. 2). His mother likewise showed skin lesions typical of Darier's disease, and, by history, her sister had the References 1. Supplied by Dr. Leon Warren, Parke Davis & Company, Detroit. 2. Supplied by Upjohn Co., Kalamazoo, Mich. 3. Supplied by Mr. J. R. Lucas, Winthrop Laboratories, New York, N. Y. 4. Supplied by Dr. Nicholas Capeci, Merck Sharp & Dohme, West Point, Pa. 5. Supplied by Dr. Gavin Hildick-Smith, Johnson & Johnson, New Brunswick, N. J. 6. Supplied by Dr. Christopher Demos, Lederle Laboratories, Pearl River, N. Y. 7. Miani, G.: La malattia di Darier allo stato attuale delle nostre Conascenze , Arch. ital. dermat. sif. 15:433-510, 1939. 8. Hambrick, G. W., and Leedham, C. L.: Keratosis Follicularis or Darier's Disease: A Report of a Family , Virginia M. Month. 77:178, 1950. 9. Graham, J. H., and Helwig, E. B.: Isolated Dyskeratosis Follicularis , A. M. A. Arch. Dermat. 77:377, 1958.Crossref 10. Beard, J. W.: Viruses as a Cause of Cancer , Am. Sc. 46:226, 1958. 11. Beerman, H.: Hypertrophic Darier's Disease and Nevus Syringocystadenomatosus Papilliferus , Arch. Dermat. & Syph. 60:500, 1949. 12. Sutton, R. L., Jr.: Early Epidermal Neoplasia: Description and Interpretation: The Theory of Mutation in the Origin of Cancer , Arch. Dermat. & Syph. 37:737, 1938. 13. Berenblum, J.: Carcinogenesis and Tumor Pathogenesis , Advances Cancer Res. 2:129, 1954. 14. Cowdry, E. V.: Epidermal Carcinogenesis , Advances Cancer Res. 1:57, 1953. 15. Wolf, G.: Chemical Induction of Cancer , London, Cassell & Co., Ltd., 1952. 16. Heger, I.: Carcinogenic Substances in Human Tissues , Brit. M. Bull. 4:360, 1947. 17. Hartwell, J. L.: Survey of Compounds Which Have Been Tested for Carcinogenic Activity , Ed. 2, U. S. Public Health Service Publication, 1951 18. Supp. 1, 1957. 19. Haven, F. L., and Bloon, W. R.: Lipids in Cancer , Advances Cancer Res. 4:238, 1956. 20. Lorincz, A. L., and Stoughton, R. B.: Specific Metabolic Processes of Skin , Physiol. Rev. 38:481, 1958. 21. Klinken-Rasmussen, L.: Hair Cycle and Experimental Carcinogenesis in Mouse Skin , Copenhagen, Universitetsforlaget i Aarhus, 1956. 22. Cottini, G. B., and Mazzone, G. B.: The Effects of 3, 4 Benzopyrene on Human Skin , Am. J. Cancer 37:186, 1939. 23. Williams, M. G.: The Response of the Human Epidermis to the Application of Carcinogenic Hydrocarbons , J. Invest. Dermat. 30:13, 1958. 24. Brünauer, S. R.: Über Schleimhaut Veränderungen bei Morbus Darier und über die Pathogenese dieser Erkrankung , Acta dermat.-venereol. 6:131, 1925. 25. Bettmann, S.: Über Umbauvorgange als Ausdruck spezifischer Reaktionsfähigkeit bei Hautkrankheiten (Die Reizbarkeit der Haut bei der Darierschen Krankheit) , Arch. Dermat. u. Syph. 135:65, 1921. 26. Preissman, M.: Sur l'Action provocatrice de la lumière dans la maladie de Darier , Dermatologica 91:28, 1945. 27. Leitner, Z. A., and Moore, T.: Vitamin A in Darier's Disease , Brit. J. Dermat. 60:41, 1948. 28. Sutton, R. L., Jr., in discussion on Mooney, T. L.: Darier's Disease , A. M. A. Arch. Dermat. 72:590, 1955. 29. Stoltz, E.: Zur Grenzstrahlentherapie bei Morbus Darier , Dermat. Wchnschr. 105:1266, 1937. 30. Blank, H.: Keratosis Follicularis (Darier's Disease) Treated with Grenz Rays , A. M. A. Arch. Derm. & Syph. 65:634, 1952. 31. Upton, A. C.: The Radiobiology of the Cancer Cell , Fed. Proc. 17:698, 1958.
A.M.A. Archives of Dermatology – American Medical Association
Published: Sep 1, 1959
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