Advances in Genetics in Dermatology

  • Howard P. Baden
  • Phillip A. Hooker
Chapter

Abstract

The spectacular growth of cellular and molecular biology in the past two decades has led to important advances in our understanding of many diseases. This also has been true for disorders of the skin, most particularly those with a clear genetic basis. This review will consider a number of genodermatoses in which significant progress has been made or is about to be realized. In some of these diseases the skin appears to be uniquely involved, while in others the abnormality is widespread, affecting many organs. The organization of this chapter does not imply any fundamental difference in pathogenesis, but is merely a convenient way of approaching the disorders.

Keywords

Stratum Corneum Phytanic Acid Epidermolysis Bullosa Xeroderma Pigmentosum Nevoid Basal Cell Carcinoma Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Abele, D. C., Dobson, R. L., and Graham, J. B., 1963, Heredity and psoriasis: Study of a large family, Arch. Dermatol. 88: 38–47.PubMedCrossRefGoogle Scholar
  2. Abrahams, P. J., and Eb, A. J., von der, 1976, Host-cell reactivation of ultraviolet-irradiated SV40 DNA in five complementation groups of xeroderma pigmentosum, Mutat. Res. 35: 13–22.PubMedCrossRefGoogle Scholar
  3. Ackerman, A. B., 1970, Histopathologic concept of epidermolytic hyperkeratosis, Arch. Dermatol. 102: 253–259.PubMedCrossRefGoogle Scholar
  4. Adachi, K., Iizuka, H., Halprin, K. M., and Levine, V., 1980, Epidermal cyclic AMP is not decreased in psoriasis lesions, J. Invest. Dermatol. 74: 74–76.PubMedGoogle Scholar
  5. Adams, R. D., 1979, Neurocutaneous diseases, in: Dermatology in General Medicine, 2nd ed. (T. B. Fitzpatrick, A. Z. Eisen, K. Wolff, I. M. Freedberg, and K. F. Austen, eds.), pp. 1206–1246, McGraw-Hill, New York.Google Scholar
  6. Adler, A., and Safai, B., 1979, Immunity in wart resolution, J. Am. Acad. Dermatol. 1: 305–309.PubMedCrossRefGoogle Scholar
  7. Almeida, J., Mowatson, A., and Williams, M., 1962, Electron microscopic study of human warts: Site of virus production and nature of inclusion bodies, J. Invest. Dermatol. 38: 337–345.PubMedGoogle Scholar
  8. Andrews, A. D., Barrett, S. F., and Robbins, J. H., 1978a, The relationship between neurologic disease, acute sun sensitivity, and post-ultraviolet colony-forming ability in xeroderma pigmentosum, J. Supramol. Struct. Suppl. 2: 29.Google Scholar
  9. Andrews, A. D., Barrett, S. F., and Robbins, J. H., 1978b, Xeroderma pigmentosum neurological abnormalities correlate with colony-forming ability after ultraviolet radiation, Proc. Natl Acad. Sci. USA 75: 1984–1988.PubMedCrossRefGoogle Scholar
  10. Anton-Lamprecht, I., 1978, Electron microscopy in the early diagnosis of genetic disorders of the skin, Dermatologica 157: 65–85.PubMedCrossRefGoogle Scholar
  11. Anton-Lamprecht, I., and Hashimoto, I., 1976, Epidermolysis bullosadystrophica dominans (Pasini)—a primary structural defect of the anchoring fibrils, Hum. Genet. 32: 69–76.PubMedCrossRefGoogle Scholar
  12. Anton-Lamprecht, I., and Schnyder, U. W., 1973, Epidermolysis bullosa dystrophica dominans—ein defekt der anchoring fibrils?, Dermatologica 147: 289–298.PubMedCrossRefGoogle Scholar
  13. Anton-Lamprecht, I., and Schnyder, U. W., 1974, Ultrastructure of inborn errors of keratinization. VI. Inherited ichthyoses—a model system for heterogeneities in keratinization disturbances, Arch. Dermatol. Forsch. 250: 207–227.PubMedGoogle Scholar
  14. Arase, S., Kozuka, T., Tanaka, K., Ikenaga, M., and Takebe, H., 1979, A sixth complementation group in xeroderma pigmentosum. Mutat. Res. 59: 143–146.PubMedCrossRefGoogle Scholar
  15. Arwill, T., Bergenholtz, A., and Thilander, H., 1968, Epidermolysis bullosa hereditaria. 5. The ultrastructure of oral mucosa and skin in four cases of the letalis form, Acta Pathol. Microbiol. Stand. 74: 311–324.CrossRefGoogle Scholar
  16. Baden, H. P., and Alper, J. C., 1973, A keratolytic gel containing salicylic acid in propylene glycol, J. Invest. Dermatol. 61: 330–333.PubMedCrossRefGoogle Scholar
  17. Baden, H. P., and Goldsmith, L. A., 1973, The structural proteins of the harlequin fetus: Stratum corneum, J. Invest. Dermatol. 61: 25–26.CrossRefGoogle Scholar
  18. Baden, H. P., and Lee, L. D., 1977, The structure of epidermal keratin, in: Biochemistry of Cutaneous Epidermal Differentiation (M. Seiji and I. A. Bernstein, eds.), pp. 478–492, University of Tokyo, Tokyo.Google Scholar
  19. Baden, H. P., and Lee, L. D., 1978, Fibrous protein of human epidermis, J. Invest. Dermatol. 71: 148–151.PubMedCrossRefGoogle Scholar
  20. Baden, H. P., Goldsmith, L. A., and Fleming, B., 1973a, The polypeptide composition of epidermal prekeratin, Biochim. Biophys. Acta 317: 303–311.PubMedGoogle Scholar
  21. Baden, H. P., Goldsmith, L. A., and Fleming, B., 1973b, A comparative study of the physicochemical properties of human keratinized tissues, Biochim. Biophys. Acta 322: 269–278.PubMedGoogle Scholar
  22. Baden, H. P., Lee, L. D., and Kubilus, J., 1975, A genetic electrophoretic variant of human hair a polypeptides, Am. J. Hum. Genet. 27: 472–477.PubMedGoogle Scholar
  23. Baden, H. P., Lee, L. D., and Kubilus, J., 1976a, The fibrous protein of stratum corneum, J. Invest. Dermatol. 67: 573–576.PubMedCrossRefGoogle Scholar
  24. Baden, H. P., Jackson, C. E., Weiss, L., Jimbow, K., Lee, L. D., Kubilus, J., and Gold, R. J. M., 1976b, The physicochemical properties of hair in the BIDS syndrome (brittle hair, intellectual impairment, decreased fertility and short stature), Am. J. Hum. Genet. 28: 514–521.PubMedGoogle Scholar
  25. Baden, H. P., McGilvray, N., Cheng, C. K., Lee, L. D., and Kubilus, J., 1978, The keratin polypeptides of psoriatic epidermis, J. Invest. Dermatol. 70: 294–297.PubMedCrossRefGoogle Scholar
  26. Baden, H. P., Kubilus, J., and Argyris, T. S., 1980a, Modification of polypeptide composition in keratinocyte fibrous protein, J. Invest. Dermatol. 75(4): 311–315.PubMedCrossRefGoogle Scholar
  27. Baden, H. P., Hooker, P. A., Kubilus, J., and Tarascio, A., 1980b, Sulfatase activity of keratinizing tissues in X-linked ichthyosis, Pediatr. Res. 14: 1347–1348.PubMedCrossRefGoogle Scholar
  28. Baker, H., and Ryan, T. J., 1968, Generalized pustular psoriasis: a clinical and epidemiological study of 104 cases, Br. J. Dermatol. 80: 771–792.PubMedCrossRefGoogle Scholar
  29. Ball, R. D., Walker, G. K., and Bernstein, I. A., 1978, Histidine-rich proteins as molecular markers of epidermal differentiation, J. Biol. Chem. 253(16): 5861–5868.PubMedGoogle Scholar
  30. Barakat, A. Y., and Cochran, W. E., 1978, Tuberous sclerosis: Report of nine cases and a review, Clin. Pediatr. 17: 875–879.CrossRefGoogle Scholar
  31. Barry, J. F., Harwood-Nash, C. R., and Byrd, S. E., 1977, Unrecognized atypical tuberous sclerosis diagnosed with CT, Neuroradiology 13: 177–180.PubMedCrossRefGoogle Scholar
  32. Bauer, E. A., 1977, Recessive dystrophic epidermolysis bullosa: evidence for an altered collagenase in fibroblast cultures, Proc. Natl Acad. Sci. USA 74: 4646–4650.PubMedCrossRefGoogle Scholar
  33. Bauer, E. A., and Briggaman, R. A., 1979, The meehanobullous diseases (epidermolysis bullosa), in: Dermatology in General Medicine, 2nd ed. (T. B. Fitzpatrick, A. Z. Eisen, K. Wolff, I. M. Freedberg, and K. F. Austen, eds.), pp. 334–347, McGraw-Hill, New York.Google Scholar
  34. Bauer, E. A., and Eisen, A. Z., 1978, Recessive dystrophic epidermolysis bullosa. Evidence for increased collagenase as a genetic characteristic in cell culture, J. Exp. Med. 48: 1378–1387.CrossRefGoogle Scholar
  35. Bauer, E. A., Gedde-Dahl, T., and Eisen, A. Z., 1977, The role of human skin collagenase in epidermolysis bullosa, J. Invest. Dermatol. 68: 119–124.PubMedCrossRefGoogle Scholar
  36. Bauer, E. A., Fiehler, W. K., and Esterly, N. B., 1979, Increased glycosaminoglycan accumulation as a genetic characteristic in cell cultures of one variety of dominant dystrophic epidermolysis bullosa, J. Clin. Invest. 64: 32–39.PubMedCrossRefGoogle Scholar
  37. Bauer, E. A., Cooper, T. W., Tucker, D. R., and Esterly, N. B., 1980, Diphenylhydantoin therapy of recessive dystrophic epidermolysis bullosa: Clinical trial and proposed mechanism of action on collagenase, New Eng. J. Med. 303(14): 776–781.PubMedCrossRefGoogle Scholar
  38. Benedict, P. H., Szabo, G., Fitzpatrick, T. B., and Sinesi, S. J., 1968, Melanotic macules in Albright’s syndrome and in neurofibromatosis, JAMA 205: 618–626.PubMedCrossRefGoogle Scholar
  39. Bentley-Phillips, B., and Bayles, M. A., 1973, A previously undescribed hereditary hair anomaly (pseudo-monilethrix), Br. J. Dermatol. 89: 159–167.PubMedCrossRefGoogle Scholar
  40. Bienfang, D. C., Kuwabara, T., and Pueschel, S. M., 1976, The Richner-Hanhart syndrome. Report of a case with associated tyrosinemia, Arch. Ophthal. 94: 1133–1137.PubMedCrossRefGoogle Scholar
  41. Binkley, G. W., and Johnson, H. H., Jr., 1951, Epithelioma adenoides cysticum: basal cell nevi, agenesis of corpus callosum and dental cysts, Arch. Derm. Syph. (Chic.) 63: 73–84.CrossRefGoogle Scholar
  42. Black, R. L., 1979, Psoriatic arthritis, in: Dermatology in General Medicine, (T. B. Fitzpatrick, A. Z. Eisen, K. Wolff, I. M. Freedburg, and K. F. Austen eds.), pp. 248–252, McGraw-Hill, New York.Google Scholar
  43. Blair, C., 1976, The action of a urea lactic acid ointment in ichthyosis with particular reference to the thickness of the horny layer, Br. J. Dermatol. 94: 145–153.PubMedCrossRefGoogle Scholar
  44. Blanchet-Bardon, Cl., Anton-Lamprecht, A., Puissant, A., and Schnyder, U. W., 1978, Ultrastructural features of ichthyotic skin in Refsum’s syndrome, in: The Ichthyoses (R. Marks and P. J. Dykes, eds.), pp. 65–69, Spectrum Publications, New York.Google Scholar
  45. Block, J. B., and Clendenning, W. E., 1963, Parathyroid hormone hyporesponsiveness in patients with basal-cell nevi and bone defects, New Eng. J. Med. 268: 1157–1162.PubMedCrossRefGoogle Scholar
  46. Bourneville, D. 1880, Scléreuse tubéreuse des circonvolutions cerébrales, Idiotie et épilepsie hémiplégique, Arch. Neurol. 1: 81–91.Google Scholar
  47. Boyer, B. E., and Martin, M. D., 1958, Marfan’s syndrome: report of case manifesting giant bone of cyst of mandible and multiple (110) basal cell carcinomata, Plast. Reconstr. Surg. 22: 257–263.CrossRefGoogle Scholar
  48. Braverman, I. M., 1977, Microcirculation in psoriasis, in: Psoriasis: Proceedings of the Second International Symposium (E. M. Färber, and A. J. Cox, eds.), pp. 828–835, Yorke Med. Books, New York.Google Scholar
  49. Breathnach, A. S., and Wolff, K. 1979, Structure and development of the skin, in: Dermatology in Modern Medicine, Second edition, (T. B. Fitzpatrick, A. Z. Eisen, K. Wolff, I. M. Freedberg, and K. F. Austen, eds.), pp. 41–59, McGraw-Hill, New York.Google Scholar
  50. Briggaman, R. A., and Wheeler, C. E., 1975, Epidermolysis bullosa dystrophica-recessive: a possible role of anchoring fibrils in the pathogenesis, J. Invest. Dermatol. 65: 203–211.PubMedCrossRefGoogle Scholar
  51. Briggaman, R. A., and Wheeler, C. E., 1976, Lamellar ichthyosis: Long term graft studies on congenitally athymic mice, J. Invest. Dermatol. 67: 567–572.PubMedCrossRefGoogle Scholar
  52. Briggaman, R. A., and Wheeler, C. E., 1979, Immunology of human warts, J. Am. Acad. Dermatol. 1: 297–304.PubMedCrossRefGoogle Scholar
  53. Brodersen, I., Genner, J., and Brodthagen, H., 1974, Tuberculin sensitivity in BCG-vaccinated children with common warts, Acta Derm. Venereol. (Stockh.) 54: 291–292.Google Scholar
  54. Brody, I., 1959, An ultrastructural study on the role of the keratohyalin granules in the keratinization process, J. Ultrastruct. Res. 3: 84–104.PubMedCrossRefGoogle Scholar
  55. Brody, I., 1968, An electron-microscopic study of the junctional and regular desmosomes in normal human epidermis, Acta Derm. Venereol. 48: 290–302.PubMedGoogle Scholar
  56. Brown, A. C., Belser, R. B., Crounse, R. G., and Wehr, B. F., 1970, A congenital hair defect: Trichoschisis with alternating birefringence and low sulfur content, J. Invest. Dermatol. 54: 496–509.PubMedCrossRefGoogle Scholar
  57. Bucknall, W. E., Haslam, R. H. A., and Holtzman, N. A., 1973, Kinky hair syndrome: response to copper therapy, Pediatr. 52: 653–657.Google Scholar
  58. Buist, N. R. M., Kennaway, N. G., and Fellman, J. H., 1974, Disorders of tyrosine metabolism, in: Inheritable Disorders of Amino Acid Metabolism (W. L. Nyhan, ed.), pp. 160–176, Wiley, New York.Google Scholar
  59. Burk, P. G., Yuspa, S. H., Lutzner, M. A., and Robbins, J. H., 1971a, Xeroderma pigmentosum and DNA repair, Lancet 1: 601.PubMedCrossRefGoogle Scholar
  60. Burk, P. G., Lutzner, M. A., Clarke, D. D., and Robbins, J. H., 1971b, Ultraviolet-stimulated thymidine incorporation in xeroderma pigmentosum lyphocytes, J. Lab. Clin. Med. 77: 759–767.PubMedGoogle Scholar
  61. Burns, R. P., 1972, Soluble tyrosine aminotransferase deficiency: An unusual cause of corneal ulcers, Am. J. Ophthalmol. 73: 400–402.PubMedGoogle Scholar
  62. Buxman, M. M., Goodkin, P. E., Fahrenbach, W. H., and Dimond, R. L., 1979, Harlequin ichthyosis with epidermal lipid abnormality, Arch. Dermatol. 115: 189–193.PubMedCrossRefGoogle Scholar
  63. Callan, N. J., 1970, Circumscribed palmoplantar keratoderma, Aust. J. Dermatol. 11: 76–81.CrossRefGoogle Scholar
  64. Calnan, C. D., 1953, Two cases of multiple naevoid basal cell epitheliomata? Porokeratosis of Mantoux, Br. J. Dermatol. 65: 219.Google Scholar
  65. Canale, D., Bebin, J., and Knighton, R. S., 1964, Neurologic manifestations of von-Recklinghausen’s disease of the nervous system, Confin. Neurol. 24: 359–403.PubMedCrossRefGoogle Scholar
  66. Carnes, W. M., 1971, Role of copper in connective tissue metabolism, Fed. Proc. 30: 995–1000.PubMedGoogle Scholar
  67. Carney, R. G., 1952, Linear unilateral basal-cell nevus with comedones. Report of case, Arch. Derm. Syph. (Chic.) 65: 471–476.CrossRefGoogle Scholar
  68. Chandra, R. K., 1980, Acrodermatitis enteropathica: Zinc levels and cell-mediated immunity, Pediatr. 66(5): 789–791.Google Scholar
  69. Chernosky, M. E., 1974, Acquired trichorrexis nodosa, in: The First Human Hair Symposium, (A. C. Brown, ed.), pp. 36–49, Medcom Press, New York.Google Scholar
  70. Chernosky, M. E., and Owens, P., 1966, Trichorrhexis nodosa. Clinical and investigative studies, Arch. Dermatol. 94: 577–585.PubMedCrossRefGoogle Scholar
  71. Chretien, J., Esswein, J., and Garagusi, V., 1978, Decreased T cell levels in patients with warts, Arch. Dermatol. 114: 213–215.PubMedCrossRefGoogle Scholar
  72. Christensen, K., Fischer, P., Knudsen, K. E. B., Larsen, S., Sorensen, H., and Venge, O., 1979, A syndrome of hereditary tyrosinemia in mink, Can. J. Comp. Med. 43: 333–340.PubMedGoogle Scholar
  73. Christophers, E., Wolff, H. H., and Laurence, E. B., 1974, The formation of epidermal cell columns, J. Invest. Dermatol. 62: 555–559.PubMedCrossRefGoogle Scholar
  74. Cleaver, J. E., 1968, Defective repair replication of DNA in xeroderma pigmentosum, Nature 218: 652–656.PubMedCrossRefGoogle Scholar
  75. Cleaver, J. E., 1972, Xeroderma pigmentosum: variants with normal DNA repair and normal sensitivity to ultraviolet light, J. Invest. Dermatol. 58: 124–128.PubMedCrossRefGoogle Scholar
  76. Cleaver, J. E., 1977, DNA repair processes and their impairment in some human diseases, in: Progress in Genetic Toxicology (D. Scott, B. A. Bridges, and F. H. Sobels, eds.), pp. 29–42, Elsevier/North-Holland Biomedical Press, New York.Google Scholar
  77. Cleaver, J. E., 1980, DNA damage, repair systems and human hypersensitive diseases, J. Environm. Pathol. Toxicol. 3: 53–68.Google Scholar
  78. Cleaver, J. E., and Bootsma, D., 1975, Xeroderma pigmentosum: biochemical and genetic characteristics, Annu. Rev. Genet. 9: 19–38.PubMedCrossRefGoogle Scholar
  79. Cleaver, J. E., and Painter, R. B., 1968, Evidence for repair replication of HeLa cell DNA damaged by ultraviolet light, Biochim. Biophys. Acta 161: 552–554.PubMedGoogle Scholar
  80. Cockayne, E. A., Inherited Abnormalities of the Skin and its Appendages, Oxford University Press, London.Google Scholar
  81. Collie, W. R., Hefferman, R. M., Arbisser, A., and Howell, R. R., in press, Sabinas syndrome, in: Hair, Trace Elements and Human Illness (A. C. Brown and R. G. Crounse, eds.), Praeger Publishers, New York.Google Scholar
  82. Comaish, S., 1969, Autoradiographic studies of hair growth and rhythm in monilethrix, Br. J. Dermatol. 81: 443–447.PubMedCrossRefGoogle Scholar
  83. Craig, J. M., Goldsmith, L. A., and Baden, H. P., 1970, An abnormality of keratin in the harlequin fetus, Pediatr. 46(3): 437–440.Google Scholar
  84. Crawford, E. G., Jr., Burkes, E. J., Jr., and Briggaman, R. A., 1976, Hereditary epidermolysis bullosa: oral manifestations and dental therapy, Oral Surg., Oral Med., Oral Pathol. 42(4): 490–500.CrossRefGoogle Scholar
  85. Crewther, W. G., Fraser, R. D. B., Lennox, F. G., and Lindley, H., 1965, The chemistry of keratins, in: Advances in Protein Chemistry, Vol. 20 (C. B. Anflnsen, Jr., M. L. Anson, J. T. Edsall, and R. M. Richards, eds.), p. 191, Academic Press, New York.Google Scholar
  86. Crowe, F. W., 1964, Axillary freckling as a diagnostic aid in neurofibromatosis, Ann. Intern. Med. 61: 1142–1143.PubMedGoogle Scholar
  87. Crowe, F. W., Schull, W. J., and Neel, J. V., 1956, A Clinical, Pathological and Genetic Study of Multiple Neurofibromatoses, pp. 1–181, C. C Thomas, Springfield, Illinois.Google Scholar
  88. Cutler, L. E., Bernstein, J. E., and Soltani, K., 1980, Trizygotic triplets and dizygotic twins in a family with psoriasis, Arch. Dermatol. 116: 718–719.PubMedCrossRefGoogle Scholar
  89. Dale, B. A., 1977, Purification and characterization of a basic protein from the stratum corneum of mammalian epidermis, Biochim. Biophys. Acta 491: 193–204.PubMedGoogle Scholar
  90. Dale, B. A., Holbrook, K. A., and Steinert, P. M., 1978, Assembly of stratum corneum basic protein and keratin filaments in macrofibrils, Nature 276: 729–731.PubMedCrossRefGoogle Scholar
  91. Danbolt, N., and Closs, K., 1943, Acrodermatitis enteropathica, Acta Derm. Venereol. (Stockh.) 23: 127–169.Google Scholar
  92. Danks, D. M., Campbell, P. E., Stevens, B. J., Mayne, V., and Cartwright, E., 1972a, Menkes’ kinky hair syndrome; an inherited defect in copper absorption with widespread effects, Pediatr. 50: 188–201.Google Scholar
  93. Danks, D. M., Campbell, P. E., Walker-Smith, J., Stevens, B. J., Gillespie, J. M., and Blomfield, J., 1972b, Menkes’ kinky hair syndrome, Lancet 1: 1101–1103.Google Scholar
  94. Danks, D. M., Campbell, P. E., Cartwright, E. C., Gillespie, J. M., Townely, R. R. W., Walker-Smith, J. A., Blomfîeld, J., Turner, B. B., and Mayne, V., 1974, Menkes’ kinky-hair syndrome. An inherited defect in the intestinal absorption of copper with widespread effects, Birth Defects: Original Article Series 10(10): 132–137.Google Scholar
  95. Dawber, R., and Comaish, S., 1970, Scanning electron microscopy of normal and abnormal hair shafts, Arch. Dermatol. 101: 316–322.PubMedCrossRefGoogle Scholar
  96. Debard, A., and Richardet, J. M., 1975, Signification des taches ochromiques chez le nourrison, Nouv. Presse Med. 33: 2404–2415.Google Scholar
  97. De Bruÿn, H. W. A., Rijken, Y., Meijer, A. E. F. H., and Marinkovic-Ilsen, A., 1980, X-linked ichthyosis and X-linked placental sulfatase deficiency: A disease entity, Am. J. Pathol. 99: 279–286.Google Scholar
  98. Deck, G. L., 1980, Retinoids in dermatology. An interim report, Arch. Dermatol. 116: 283–284.CrossRefGoogle Scholar
  99. Deraemarker, R., 1957, Monilethrix: Report of a family with special reference to some problems concerning inheritance, Am. J. Hum. Genet. 9(3): 195–199.Google Scholar
  100. DeSanctis, C., and Cacchione, A., 1932, L’idiozia xerodermica, Riv. Sper. Freniatr. 56: 269–292.Google Scholar
  101. de Weerd-Kastelein, E. A., Keijzer, W., and Bootsma, D., 1972, Genetic heterogeneity of xeroderma pigmentosum demonstrated by somatic cell hybridization, Nature 238: 75–76.Google Scholar
  102. de Weerd-Kastelein, E. A., Keijzer, W., and Bootsma, D., 1974, A third complementation group in xeroderma pigmentosum, Mutat. Res. 22: 87–91.PubMedCrossRefGoogle Scholar
  103. Dicken, C. H., and Connoly, S. M., 1980, Eruptive xanthomas associated with isotretinoin (13-cis-retinoic acid), Arch. Dermatol. 116: 951–952.PubMedCrossRefGoogle Scholar
  104. Dillaha, C. J., and Lorincz, A. L., 1953, Enteropathic acrodermatitis (danbolt): Successful treatment with diodoquin (diiodohydroxyquinoline), Arch. Dermatol. 67: 324–326.Google Scholar
  105. Dobson, R. L., 1980, The inheritance of psoriasis, Arch. Dermatol. 116: 657.PubMedCrossRefGoogle Scholar
  106. Dykes, P. J., Marks, R., Davies, M. G., and Reynolds, D. J., 1978, Epidermal metabolism in heredopathia atatica polyneuritiformis (Refsum’s disease), J. Invest. Dermatol. 70: 126–219.PubMedCrossRefGoogle Scholar
  107. Eckhert, C. D., Sloan, M. V., Duncan, J. R., and Hurley, I. S., 1977, Zinc binding: difference between human and bovine milk, Science 195: 789–790.PubMedCrossRefGoogle Scholar
  108. Edmonds, H. W., and Dolan, W. D., 1957, Ichthyosis congenita fetalis, severe type (harlequin fetus), Bull. Int. Assoc. Med. Mus. 32: 1.Google Scholar
  109. Efron, M. L., and Hoefnagel, D., 1966, Argininosuccinic acid in monilethrix, Lancet 1: 321–322.PubMedCrossRefGoogle Scholar
  110. Eisen, A. Z., 1969, Human skin collagenase: relationship to the pathogenesis of epidermolysis bullosa dystrophica, J. Invest. Dermatol. 52: 449–453.PubMedGoogle Scholar
  111. Eisenberg, M., Williams, J. F., Stevens, L., and Schofield, P. J., 1974, Mammalian collagenase and peptidase estimation in normal skin and in the skin of patients suffering from epidermolysis bullosa, J. Int. Res. Comm. 2: 1732.Google Scholar
  112. Eisenberg, M., Stevens, L., and Schofield, P. J., 1978, Epidermolysis bullosa—new therapeutic approaches. Aust. J. Dermatol. 19: 1–8.CrossRefGoogle Scholar
  113. Eldridge, R., 1980, Clinical neurogenetics: Needs versus resources, Neurol. 30: 860–863.Google Scholar
  114. Elias, P. M., Goerke, J., and Frend, D. S., 1977, Mammalisn epidermal barrier layer lipids: composition and influence on structure, J. Invest. Dermatol. 69: 535–546.PubMedCrossRefGoogle Scholar
  115. Elias, S., Mazur, M., Sabbagha, R., Esterly, N. B., and Simpson, J. L., 1980, Prenatal diagnosis of harlequin ichthyosis, Clin. Genet. 17: 275–280.PubMedCrossRefGoogle Scholar
  116. Emory, A. E. H., and Rimoin, D. L., eds., 1981, Principles and Practice of Medical Genetics, Churchill Livingston, Edinburgh.Google Scholar
  117. Endo, M., Yamamoto, R., Yosizawa, Z., Sasai, Y., and Saito, N., 1974, Urinary chondroitin of epidermolysis bullosa dystrophica et albo-papuloidea (Pasini), Clin. Chim. Acta 57: 249–253.PubMedCrossRefGoogle Scholar
  118. Epstein, E. H., Jr., and Leventhal, M. E., 1979, Detection of steroid sulfatase deficiency in peripheral blood leukocytes of patients with X-linked ichthyosis, Presented at Western Section Dermatology, AFCR.Google Scholar
  119. Fabricant, R. N., Todaro, G. T., and Eldridge, R. E., 1979, Increased levels of a nerve growth factor cross reacting protein in central neurofibromatosis, Lancet, 1: 4–7.PubMedCrossRefGoogle Scholar
  120. Farber, E. M., and Peterson, J. B., 1961, Variations in the natural history of psoriasis, Calif, Med. 95: 6–11.PubMedGoogle Scholar
  121. Farber, E. M., Grauer, F., and Zaruba, F., 1965, Racial incidence of psoriasis, Cesk. Derm. 40: 289–297.PubMedGoogle Scholar
  122. Farber, E. M., Bright, R. D., and Nall, M. L., 1968, Psoriasis—a questionnaire survey of 2144 patients, Arch. Dermatol. 98: 248–259.PubMedCrossRefGoogle Scholar
  123. Farber, E. M., Nall, M. L., and Watson, W., 1974, Natural history of psoriasis in 61 twin pairs, Arch. Dermatol. 109: 207–211.PubMedCrossRefGoogle Scholar
  124. Feinstein, A., Ackerman, A. B., and Ziprkowski, L., 1970, Histology of autosomal dominant ichthyosis vulgaris and X-linked ichthyosis, Arch. Dermatol. 101: 524–527.PubMedCrossRefGoogle Scholar
  125. Fellman, J. H., Vanbellinghen, R. J., Jones, R. T., and Koler, R. D., 1969, Soluble and mitochondrial forms of tyrosine aminotransferase. Relationship to human tyrosinemia, Biochemistry 8: 615–622.PubMedCrossRefGoogle Scholar
  126. Fellman, J. H., Fujita, T. S., and Roth, E. S., 1972, Assay, properties and tissue distribution of p-hydroxyphenylpyruvate hydroxylase, Biochim. Biophys. Acta 284: 90–100.PubMedGoogle Scholar
  127. Fischer, T., and Gedde-Dahl, T., Jr., 1979, Epidermolysis bullosa simplex and mottled pigmentation: A new dominant syndrome 1. Clinical and histological features, Clin. Genet. 15: 228–238.PubMedCrossRefGoogle Scholar
  128. Fitch, N., Segool, R., Ferenczy, A., and Cohen, H., 1976, Dominant ichthyosis vulgaris with an ultrastructurally normal granular layer, Clin. Genet. 9: 71–76.PubMedCrossRefGoogle Scholar
  129. Fitzpatrick, T. B., Szabo, G., Hori, Y., Simone, A. A., Reed, W. B., and Greenberg, M. H., 1968, White leaf-shaped macules, earliest visible sign of tuberous sclerosis, Arch. Dermatol. 98: 1–6.PubMedCrossRefGoogle Scholar
  130. Fitzpatrick, T. B., Hori, Y., Toda, K., Kenebuchi, S., and Szabo, G., 1971, The mechanism of normal human melanin pogmentation and of some pigmentary disorders, in: Biology of Normal and Abnormal Melanocytes (T. Kawamura, T. B. Fitzpatrick, and M. Seiji, eds.), pp. 369–401, University Park Press, Baltimore.Google Scholar
  131. France, J. T., and Liggins, G. C., 1969, Placental sulfatase deficiency, J. Clin. Endocrinol. Metab. 29: 138–141.PubMedCrossRefGoogle Scholar
  132. Franceschetti, A. T., Schnyder, U. W., and Felgenhauer, W. R., 1975, Die Cornea beim Richner-Hanhart-Syndrom, Ber. Dt. Ophthal. Ges. 14: 937–941.Google Scholar
  133. Fraser, R. D. B., Macrae, T. B., and Rogers, G. E., 1972, Keratins, Their Compositions, Structure and Biosynthesis, p. 164, Charles C Thomas, Springfield, Illinois.Google Scholar
  134. French, J. H., Sherard, E. S., Lubell, H., Brotz, M., and Moore, C. L., 1972, Trichopoliodystrophy 1. Report of a case and biochemical studies, Arch. Neurol. 26: 229–244.PubMedCrossRefGoogle Scholar
  135. Friedberg, E. C., 1978, Xeroderma pigmentosum. Recent studies on the DNA repair defects, Arch. Pathol. Lab. Med. 102: 3–7.PubMedGoogle Scholar
  136. Frieden, E., 1973, The ferrous to ferric cycles in iron metabolism, Nut. Rev. 31: 41–44.CrossRefGoogle Scholar
  137. Frost, P., 1975, Disorders of cornification, in: Dermatology (S. L. Moschella, D. N. Pillsbury, and A. J. Hurley, eds.), p. 657, W. B. Saunders Co., Philadelphia, Pennsylvania.Google Scholar
  138. Frost, P., and Van Scott, E. J., 1966, Ichthyosiform dermatoses. Classification based on anatomic and biometric observations, Arch. Dermatol. 94: 113–126.PubMedCrossRefGoogle Scholar
  139. Frost, P., and Weinstein, G. D., 1968, Vitamin A acid in the therapy of scaling dermatoses, Clin. Res. 16: 255.Google Scholar
  140. Frost, P., Weinstein, G. D., and Van Scott, E. J., 1966, The ichthyosiform dermatoses. II. Autoradiographic studies of epidermal proliferation, J. Invest. Dermatol. 47: 561–567.PubMedGoogle Scholar
  141. Frost, P., Weinstein, G. D., Bothwell, J. W., and Wildnauer, R., 1968, Ichthyosiform dermatoses III. Studies of transepidermal water loss, Arch. Dermatol. 98: 230–233.PubMedCrossRefGoogle Scholar
  142. Fukuyama, K., and Epstein, W. L., 1975, A comparative autoradiographic study of keratohyalin granules containing cysteine and histidine, J. Ultrastruct. Res. 51: 314–325.PubMedCrossRefGoogle Scholar
  143. Fukuyama, K., Wier, K. A., and Epstein, W. L., 1972, Dense homogeneous deposits of keratohyalin granules in newborn rat epidermis, J. Ultrastruct. Res. 38: 16–26.PubMedCrossRefGoogle Scholar
  144. Gardner, D. G., and Hudson, C. D., 1975, The disturbances in odontogenesis in epidermolysis bullosa hereditaria lethalis, Oral Surg., Oral Med., Oral Pathol. 40(4): 483–493.CrossRefGoogle Scholar
  145. Garnica, A. D., Trias, J. L., and Rennert, O. M., 1977, Menkes kinky hair syndrome: Is it treatable?, Clin. Genet. 11: 154–161.PubMedCrossRefGoogle Scholar
  146. Gastaut, H., Gastaut, J. L., and Pinsard, N., 1976, Diagnosis of Bourneville’s disease using computer-controlled cerebral tomography, Nouv. Presse Med. 5: 864.PubMedGoogle Scholar
  147. Gauthier, R., Vigreaver, N., Blean, G., Chapdelane, A., and Roberts, R. D., 1978, Solubilization and partial purification of steroid sulfatase of human placenta steroids, Steroids 31: 786–797.CrossRefGoogle Scholar
  148. Gazit, E., Brenner, S., Efler, T., Orgad, S., Mizradin, Y., and Krakowski, A., 1978, HLA antigens in patients with psoriasis, Tissue Antigens 12: 195–199.PubMedCrossRefGoogle Scholar
  149. Gedde-Dahl, T., 1971a, Epidermolysis Bullosa. A Clinical, Genetic and Epidermiological Study, Johns Hopkins Press, Baltimore, Maryland.Google Scholar
  150. Gedde-Dahl, T., 1971b, Phenotype-genotype correlations in epidermolysis bullosa, Birth Defects: Original Article Series VII(8): 107–117.Google Scholar
  151. Gelfant, S., 1976, The cell cycle in psoriasis: a reappraisal, Br. J. Dermatol. 95: 577–590.PubMedCrossRefGoogle Scholar
  152. Genner, J., Localization of wart virus antigen in human plantar warts, Acta Derm. Venereol. (Stockh.) 51: 361–364.Google Scholar
  153. Gibberd, F. B., Page, N. G. R., Billimoria, J. D., and Retsas, S., 1979, Heredopathia atactica polyneuritiformis (Refsum’s disease) treated by diet and plasma-exchange, Lancet 1: 575–578.PubMedCrossRefGoogle Scholar
  154. Gilgor, R. S., Chiaramonti, A., Goldsmith, L. A., and Lazarus, G. S., 1980, Evaluation of 13-cis retinoic acid in lamellar ichthyosis, pityriasis rubra pilaris, and Darier’s disease, Cutis 25: 380–385.PubMedGoogle Scholar
  155. Gilhuus-Moe, O., Haugen, L. K., and Dee, P. M., 1968, The syndrome of multiple cysts of the jaws, basal cell carcinomata and skeletal anomalies, Br. J. Oral Surg. 6: 211–222.Google Scholar
  156. Gillespie, J. M., 1973, Keratin structure and changes with copper deficiency, Austr. J. Dermatol. 14: 127–131.CrossRefGoogle Scholar
  157. Gissmann, L., Pfister, H., and zur Hausen, H., 1977, Human papilloma viruses (HPV): Characterization of four different isolates, Virology 76: 569–580.PubMedCrossRefGoogle Scholar
  158. Glick, N. R., Snodgrass, P. J., and Schafer, I. A., 1976, Neonatal argininosuccinic aciduria with normal brain and kidney but absent liver argininosuccinate lyase activity, Am. J. Hum. Genet. 28: 22–30.PubMedGoogle Scholar
  159. Golbus, M. S., Sagebiel, R. W., Filly, R. A., Gindhart, T. D., and Hall, J. G., 1980, Prenatal diagnosis of congenital bullous ichthyosiform erythroderma (epidermolytic hyperkeratosis) by fetal skin biopsy, New Eng. J. Med. 302(24): 93–95.PubMedCrossRefGoogle Scholar
  160. Gold, A. P., and Freeman, J. M., 1965, Depigmented nevi, the earliest sign of tuberous sclerosis, Pediatr. 35: 1003–1005.Google Scholar
  161. Goldsmith, L. A., 1975, Hemolysis and lysosomal activation by solid state tyrosine, Biochem. Biophys. Res. Commun. 64(2): 558–565.PubMedCrossRefGoogle Scholar
  162. Goldsmith, L. A., 1976, Haemolysis induced by tyrosine crystals, Biochem. J. 158: 17–22.PubMedGoogle Scholar
  163. Goldsmith, L. G., 1978, Molecular biology and molecular pathology of a newly described molecular disease—Tyrosinemia II (The Richner-Hanhart Syndrome), Exp. Cell Biol. 46: 96–113.PubMedGoogle Scholar
  164. Goldsmith, L. A., 1981, Tyrosine aminotransferase deficiency in mink (Mustela vision): A model for human tyrosinemia II, Biochem. Genet. (accepted for publication)Google Scholar
  165. Goldsmith, L. A., and Reed, J., 1976, Tyrosine-induced eye and skin lesions. A treatable genetic disease, JAMA 236: 382–384.PubMedCrossRefGoogle Scholar
  166. Goldsmith, L. A., Kang, E., Bienfang, D. C., Jimbow, K., Gerald, P., and Baden, H. P., 1973, Tyrosinemia with plantar and palmar keratosis and keratitis, J. Pediatr. 83(5): 798–805.PubMedCrossRefGoogle Scholar
  167. Goldsmith, L. A., Thorpe, J., and Roe, C. R., 1979, Hepatic enzymes of tyrosine metabolism in tyrosinemia II, J. Invest. Dermatol. 73: 530–532.PubMedCrossRefGoogle Scholar
  168. Golitz, L. E., Norris, D. A., Luekens, C. A. Jr., and Charles, D. M., 1980, Nevoid basal cell carcinoma syndrome—multiple basal cell carcinomas of the palms after radiation therapy, Arch. Dermatol. 116: 1159–1163.PubMedCrossRefGoogle Scholar
  169. Gomez, M. R., Mellinger, J. F., and Reese, D. F., 1975, The use of computerized axial tomography in the diagnosis of tuberous sclerosis, Mayo Clin. Proc. 50: 553–556.PubMedGoogle Scholar
  170. Goodman, S. I., Mace, J. W., Turner, B., and Garrett, W. J., 1973, Antenatal diagnosis of argininosuccinic aciduria, Clin. Genet. 4: 236–240.PubMedCrossRefGoogle Scholar
  171. Gorlin, R. J., and Goltz, R. W., 1960, Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib syndrome, New Eng. J. Med. 262: 908–912.PubMedCrossRefGoogle Scholar
  172. Gorlin, R. J., and Sedano, H. O., 1971, The multiple nevoid basal cell carcinoma syndrome revisited, Birth Defects: Original Article Series VII(8): 140–148.Google Scholar
  173. Gorlin, R. J., Yunis, J. J., and Tuna, N., 1963, Multiple nevoid basal cell carcinoma, odontogenic keratocytes and skeletal anomalies syndrome, Acta Derm. Venereol. (Stockh.) 43(1): 39–55.Google Scholar
  174. Granek, H., and Baden, H. P., 1980, Corneal involvement in epidermolysis bullosa simplex, Arch. Ophthal. 98: 469–472.PubMedCrossRefGoogle Scholar
  175. Greitz, T., and Hindmarsh, T., 1974, Computer assisted tomography of intracranial CSF circulation using a water-solution contrast medium, Acta Radiol. 15: 497–507.Google Scholar
  176. Grosshans, E., and Henry, M., 1978, Skin polyamine levels in psoriasis—the effect of therapy, J. Invest. Dermatol. 70(4): 227A.Google Scholar
  177. Halprin, K. M., 1972, Epidermal “turnover time”—a re-examination, Br. J. Dermatol. 86: 114–119.CrossRefGoogle Scholar
  178. Hameister, H., Wolff, G., Lauritzen, C. H., Lehman, W. D., Hauser, A., and Ropers, H. H., 1979, Clinical and biochemical investigations on patients with partial deficiency of placental steroid sulfatase, Hum. Genet. 46: 199–207.PubMedCrossRefGoogle Scholar
  179. Hammerström, S., Hamburg, M., Samuelsson, B., Duell, E. A., Stawiski, M., and Voorhees, J. J., 1975, Increased concentrations of nonsterified arachidonic acid, prostaglandin E2 and prostaglandin F2x in epidermis of psoriasis, Proc. Natl Acad. Sci. USA 72: 5130–5134.CrossRefGoogle Scholar
  180. Hammarström, S., Hamber, M., Duell, E. A., Stawiski, M. A., Anderson, T. F., and Voorhees, J. J., 1977, Glucocorticoid in inflammatory proliferative skin disease reduces arachidonic and hydroxyeicosatetraenoic acids, Science 197: 994–995.PubMedCrossRefGoogle Scholar
  181. Hanhart, E. 1947, Neue Sonderformen von keratosis palmoplantaris, u.a. eine regelma g-dominante mit systematisierten lipomen, ferner 2 einfachreszessive mit schwachsinn und z.T. protein fractions from hair, Biochemistry 10: 624–630.Google Scholar
  182. Hashem, N., Bootsma, D., Keijzer, W., Greene, A., Coriell, L., Thomas, G., and Cleaver, J. E., 1980, Clinical characteristics, DNA repair, and complementation groups in xeroderma pigmentosum patients from Egypt, Cancer Res. 40: 13–18.PubMedGoogle Scholar
  183. Hashimoto, I., Anton-Lamprecht, I., Gedde-Dahl, T., and Schnyder, U. W., 1975, Ultra- structural studies in epidermolysis bullosa hereditaria. I. Dominant dystrophic type of Pasini, Arch. Dermatol. Forsch. 252: 167–178.PubMedGoogle Scholar
  184. Hashimoto, I., Gedde-Dahl, T., Schnyder, U. W., and Anton-Lamprecht, I., 1976a, Ultrastructural studies in epidermolysis bullosa hereditaria. II. Dominant dystrophic type of Cockayne and Touraine, Arch. Dermatol. Forsch. 255: 285–295.Google Scholar
  185. Hashimoto, I., Schnyder, U. W., Anton-Lamprecht, I., Gedde-Dahl, T., and Ward, S., 1976b, Ultrastructural studies in epidermolysis bullosa hereditaria. III. Recessive dystrophic types with dermolytic blistering (Hallopeau-Siemens types and inverse type), Arch. Dermatol. Forsch. 256: 137–150.Google Scholar
  186. Hashimoto, I., Gedde-Dahl, T., Schnyder, U. W., and Anton-Lamprecht, I., 1976c, Ultra- structural studies in epidermolysis bullosa hereditaria. IV. Recessive dystrophic types with junctional blistering, Arch. Dermatol. Forsch. 257: 17–32.Google Scholar
  187. Hellgren, L. 1967, Psoriasis—The prevalence in sex, age and occupational groups, in: Total Populations in Sweden—Morphology, Inheritance and Association with Other Skin and Rheumatic Diseases, Almquist & Wiksells, Stockholm.Google Scholar
  188. Herlitz, G., 1935, Kongenitaler, Nicht syphilitischer pemphigus. Eine Übersicht nebst beschreibung einer neuen krankheitsform, Acta Paediatr. 17: 315–371.CrossRefGoogle Scholar
  189. Hermans, E. J., Grosfeld, J. C. M., and Valk, L. E. M., 1960, Eine funfte phakomatosis; naevus epitheliomatodes multiplex, Hautartzt 11: 160–164.Google Scholar
  190. Herndon, J. H., Jr., Steinberg, D., Uhlendorf, B. W., and Fales, H. M., 1969, Refsum’s disease: Characterization of the enzyme defect in cell culture, J. Clin. Invest. 48: 1017–1032.PubMedCrossRefGoogle Scholar
  191. Hess, J. G., and Schultz, O. T., 1921, Keratosis diffusa fetalis (ichthyosis congenita), Am. J. Dis. Child. 21: 357.Google Scholar
  192. Hill, A., and Zaleski, W. A., 1971, Tyrosinosis: Biochemical studies of an unusual case, Clin. Biochem. 4: 263.PubMedCrossRefGoogle Scholar
  193. Hobbins, J. C., 1979, Diagnosing with the fetoscope, Contemp. Ob. Gyn. 13: 143–152.Google Scholar
  194. Hoffmann, E., 1926, Über verallgemeinerte Warzenerkrankung (Verrucosis generalisata) und ihre Beziehung zur Epidermodysplasia verruciformis (Lewandowsky), Derm. Z. 48: 241–266.CrossRefGoogle Scholar
  195. Holston, J. L., Levy, L. L., Tomlin, G. A., Atkins, R. J., Patton, T. H., and Hosty, T. S., 1971, Tyrosinosis: A patient without liver or renal disease, Pediatr. 48: 393–400.Google Scholar
  196. Holtzman, N. A., 1976, Menkes’ kinky hair syndrome: a genetic disease involving copper, Fed. Proc. 35: 2276–2280.PubMedGoogle Scholar
  197. Horn, N., 1976, Copper incorporation studies on cultured cells for prenatal diagnosis of Menkes’ disease, Lancet 1: 1156–1158.PubMedCrossRefGoogle Scholar
  198. Horn, N., Mikkelsen, M., Heydorn, K., Damsgaard, E., and Tygstrup, I., 1975, Copper and steely hair, Lancet 1: 1236.PubMedCrossRefGoogle Scholar
  199. Howell, J. B., and Caro, M. R., 1959, Basal-cell nevus: its relationship to multiple cutaneous cancers and associated anomalies of development, Arch. Dermatol. 79: 67–80.CrossRefGoogle Scholar
  200. Howell, J. B., and Freeman, R. G., 1980, Structure and significance of the pits with their tumors in the nevoid cell carcinoma syndrome, J. Am. Acad. Dermatol. 2: 224–238.PubMedCrossRefGoogle Scholar
  201. Howell, J. B., and Mehregan, A. H., 1970, Story of the pits—a historic vignette, Arch. Dermatol. 102: 583–597.PubMedCrossRefGoogle Scholar
  202. Hunt, J. C., and Pugh, D. G., 1961, Skeletal lesions in neurofibromatosis, Radiology 76: 1–7.PubMedGoogle Scholar
  203. Ingram, J. T., 1934, Monilethrix, Br. J. Dermatol. 46: 272–277.CrossRefGoogle Scholar
  204. Ivanyi, L., and Morison, W., 1976, In vitro lymphocyte stimulation by wart antigen in man, Br. J. Dermatol. 94: 523–527.PubMedCrossRefGoogle Scholar
  205. Jablonska, S., and Milewski, B., 1957, Zur kenntnis der epidermodysplasia verruciformis, Dermatologica 115: 1–122.PubMedCrossRefGoogle Scholar
  206. Jablonska, S., Dabrowski, J., and Jakubowicz, K., 1972, Epidermodysplasia ve-ruciformis as a model in studies on the role of papovaviruses in oncogenesis, Cancer Res. 32: 583–589.PubMedGoogle Scholar
  207. Jablonska, S., Orth, G., Jarzabek-Chorzelska, M., Rzesa, G., Obalek, S., Glinski, W., Favre, M., and Croissant, O., 1978, Immunological studies in epidermodysplasia verruciformis, Bull. Cancer 65(2): 183–190.PubMedGoogle Scholar
  208. Jablonska, S., Orth, G., Jarzabek-Chorzelska, M., Rzesa, G., Obalek, S., Glinski, W., Favre, M., and Crossant, O., 1979a, Epidermodysplasia verruciformis versus disseminated verrucae planae: Is epidermodysplasia verruciformis a generalized infection with wart virus?, J. Invest. Dermatol. 72: 114–119.PubMedCrossRefGoogle Scholar
  209. Jablonska, S., Orth, G., Jarzabek-Chorzelska, M., Glinski, W., Obalek, S., Rzesa, G., Croissant, O., and Favre, M., 1979b, Twenty-one years of follow-up studies of familial epidermodysplasia verruciformis, Dermatologica 158: 309–327.PubMedCrossRefGoogle Scholar
  210. Jackson, C. E., Weiss, L., and Watson, J. H. L., 1974, Brittle hair with short stature, intellectual impairment and decreased fertility: An autosomal recessive syndrome in an Amish kindred, Pediatr. 54: 201–207.Google Scholar
  211. Jarrett, A., 1973, Epidermal kinetics, in: The Physiology and Pathophysiology of the Skin (A. Jarrett, ed.), Vol. 1, pp. 107–108, Academic Press, London.Google Scholar
  212. Jay, B., Black, R. K., and Wells, R. S., 1968, Occular manifestations of ichthyosis, Br. J, Ophthal. 52: 217–266.CrossRefGoogle Scholar
  213. Jessen, H., 1970, Two types of keratohyalin granules, J. Ultrastruct. Res. 33: 95–115.PubMedCrossRefGoogle Scholar
  214. Jorizzo, J., Crounse, R. G., and Wheeler, C. E., 1980, Lamellar ichthyosis, dwarfism, mental retardation, and hair shaft abnormalities, J. Am. Acad. Dermatol. 2: 309–317.PubMedCrossRefGoogle Scholar
  215. Kahler, J. P., and Goldsmith, L. A., 1978, Extractable hair protein in Menkes syndrome, J. Pediatr. 92: 675–682.PubMedCrossRefGoogle Scholar
  216. Kanter, W. R., Eldridge, R., Fabricant, R., Allen, J. C., and Koerber, T., 1980, Central neurofibromatosis with bilateral acoustic neuroma: Genetic, clinical and biochemical distinctions from peripheral neurofibromatosis, Neurology 30: 851–859.PubMedGoogle Scholar
  217. Kaposi, M., 1882, Xeroderma pigmentosum, Med. Jahrb. Wien. 1882(October): 619–633; reprinted (in French) in Ann. Dermatol. Syph. (Paris) 4: 29–38 (1883).Google Scholar
  218. Kay, R. G., Tasman-Jones, C., Pybus, J., Whiting, R., and Block, H., 1976, A syndrome of acute ainc deficiency during total parenteral alimentation in man, Ann. Surg. 183: 331–340.PubMedCrossRefGoogle Scholar
  219. Keijzer, W., Jaspers, N. G. J., Abrahams, P. J., Taylor, A. M. R., Arlett, C. F., Zelle, B., Takebe, H., Kanmont, P. D. S., and Bootsma, D., 1979, A seventh complementation group in excision deficient xeroderma pigmentosum, Mutat. Res. 62: 183–190.PubMedCrossRefGoogle Scholar
  220. Kennaway, N. G., and Buist, N. R. M., 1971, Metabolic studies in a patient with hepatic cytosol tyrosine aminotransferase deficiency, Pediatr. Res. 5: 287–297.CrossRefGoogle Scholar
  221. Kenney, J. A., 1965, Management of dermatoses peculiar to Negroes, Arch. Dermatol. 91: 126–129.PubMedCrossRefGoogle Scholar
  222. Kienzler, J. L., Laurent, R., Coppey, J., Favre, M., Orth, G., Coupez, L., and Agache, P., 1979, Epidermodysplasie verruciforme Données ultrastructurales, virologiques et photobiologiques; a propos d’une observation, Ann. Dermatol. Venereol. (Paris) 106: 549–563.Google Scholar
  223. Kleijer, W. J., Lohmann, P. H., Mulder, M. P., and Bootsma, D., 1970, Repair of x-ray damage in DNA of cultivated cells from patients having xeroderma pigmentosum, Mutat. Res. 9: 517–523.PubMedCrossRefGoogle Scholar
  224. Klenk, E., and Kahlke, W., 1963, Über das Vorkommen der 3,7,11,15-Tetramethylhex-adecansäure (Phytansäure) in den Cholesterinestern und anderen Lipoidfraktionen dern Organe bei einem Krankheitsfall unbekannter Genese (Verdact auf Heredopathica atactica polyneuritiformis (Refsum’s syndrome), Hoppe-Sey1er’s Z. Physiol. Chemie 333: 133.CrossRefGoogle Scholar
  225. Koebner, H., 1886, Hereditäre anlage zur blasenbildung, Dtsch. Med. Wochenschr. 12 21–22.CrossRefGoogle Scholar
  226. Kogoj, F., 1926, Die Epidermodysplasia verruciformis, Acta Derm. Venereol. (Stockh.) 7: 170–179.Google Scholar
  227. Kohlenzer, P. J., and Miller, M. D., 1972, Lamellar ichthyosis: Recurrent trichophyton rubrum infection, familial abnormality of chemotaxis of polymorphonuclear leukocytes, Arch. Dermatol. 106: 755–766.Google Scholar
  228. Koppe, J. G., Rijken, Y., Jobsis, A. C., and Marinkovic-Ilsen, A., 1977, X-linked ichthyosis, a sulfatase deficiency, in: Proceedings of the Fifth International Conference on Birth Defects, Montreal, Quebec, 21–27 August, International Congress Series 1977, No. 426 (J. W. Littlefield, F. J. G. Ebling, and I. W. Henderson, eds.), p. 57, Excerpta Medica, Amsterdam.Google Scholar
  229. Koppe, J. G., Marinkovic-Ilsen, A., Rijken, Y., De Groot, W. P., and Jobsis, A. C., 1978, X-linked ichthyosis, Arch. Dis. Child. 53: 803–806.PubMedCrossRefGoogle Scholar
  230. Koudstaal, J., 1975, The histochemical demonstration of arylsulfatase in human tumors, Eur. J. Cancer 11: 809–813.PubMedCrossRefGoogle Scholar
  231. Kraemer, K. H., Coon, H. G., Petinga, R. A., Barrett, S. F., Rahe, A. E., and Robbins, J. H., 1975a, Genetic heterogeneity in xeroderma pigmentosum: complementation groups and their relationship to DNA repair rates, Proc. Natl Acad. Sci. USA 72: 59–63.PubMedCrossRefGoogle Scholar
  232. Kraemer, K. H., de Weerd-Kastelein, E. A., Robbins, J. H., Keijzer, W., Barrett, S. F., Petinga, R. A., and Bootsma, D., 1975b, Five complementation groups in xeroderma pigmentosum, Mutat. Res. 33: 327–340.PubMedCrossRefGoogle Scholar
  233. Kubilus, J., Tarascio, A. J., and Baden, H. P., 1979, Steroid-sulfatase deficiency in sex- linked ichthyois, Am. J. Hum. Genet. 31: 50–53.PubMedGoogle Scholar
  234. La Du, B. N., and Gjessing, L. R., 1978, Tyrosinosis and tyrosinemia, in: The Metabolic Basis of Inherited Disease, 4th ed. (J. B. Stanbury, J. B. Wyngaarden, and D. S. Frederickson, eds., pp. 256–267, McGraw-Hill, New York.Google Scholar
  235. Lakshmi, S., and Balasubramanian, A. S., 1979, Studies on the chaotropically solubilized arylsulfatase C and oestrone sulphatase of sheep brain, Biochim. Biophys. Acta 567: 184.PubMedGoogle Scholar
  236. Lazarus, G. S., 1972, Collagenase and connective tissue metabolism in epidermolysis bullosa, J. Invest. Dermatol. 58: 242–248.PubMedCrossRefGoogle Scholar
  237. Lee, A., and Eisenger, M., 1976, Cell-mediated immunity to human wart virus and wart- associated tissue antigens, Clin. Expl. Immunol. 26: 419–424.Google Scholar
  238. Lee, B. C. P., and Gawler, J., 1978, Tuberous sclerosis: Comparison of computed tomography and conventional neuroradiology, Radiology 127: 403–407.PubMedGoogle Scholar
  239. Lee, G. R., Nacht, S., Lukens, J. N., and Cartwright, G. E., 1968, Iron metabolism in copper-deficient swine, J. Clin. Invest. 47: 2058–2069.PubMedCrossRefGoogle Scholar
  240. Lee, L. D., Ludwig, K., and Baden, H. P., 1978, Matrix proteins of human hair as a tool for identification of individuals, Forensic Sci. 11: 115–121.PubMedGoogle Scholar
  241. Lehmann, A. R., Kirk-Bell, S., and Arlett, C. F., 1975, Xeroderma pigmentosum cells with normal levels of excision repair have a defect in DNA synthesis after UV-irradiation, Proc. Natl. Acad. Sci. USA 72: 219–223.PubMedCrossRefGoogle Scholar
  242. LeVine, M. J., McGilvray, N., and Baden, H. P., 1980, Effect of therapy on keratin polypeptide profiles of psoriatic epidermis, Arch. Dermatol. 116: 1028–1030.PubMedCrossRefGoogle Scholar
  243. Lewandowsky, F., and Lutz, W., 1922, Ein Fall einer bisher nicht beschriebenen Hauterkrankung (Epidermodysplasia verruciformis), Arch. Derm. Syph. 141: 193–203.CrossRefGoogle Scholar
  244. Lisch, K., 1937, Uber Beteilung der Augen, insbesondere das Vorkommen von Irisknotchen bei der Neurofibromatose (Recklinghausen), Z. Augenheilk. 93: 137–143.Google Scholar
  245. Lombeck, I., Schnippering, H. G., Ritzl, F., Feinendegen, L. E., and Bremer, H. J., 1975, Absorption of zinc in acrodermatitis enteropathica, Lancet 1: 855.PubMedCrossRefGoogle Scholar
  246. Lomholt, G., 1963, Psoriasis—Prevalence, spontaneous course and genetics—A census study on the prevalence of skin diseases on the Faroe Islands, Copenhagen, BEC Gad.Google Scholar
  247. Lott, I. T., Dipaolo, R., Raghavan, S. S., Clopath, P., Milunsky, A., Robertson, W. C., and Kanper, J. N., 1979, Abnormal copper metabolism in Menkes’ steely-hair syndrome, Pediatr. Res. 13: 845–850.PubMedCrossRefGoogle Scholar
  248. Lutz, W., 1946, A propos de l’epidermodysplasie verruciforme, Dermatologica 92: 30–47.PubMedCrossRefGoogle Scholar
  249. Lytle, C. D., Aaronson, S. A., and Harvey, E., 1972, Host-cell reactivation in mammalian cells. II. Survival of herpes simplex virus and vaccinia virus in normal human and xeroderma pigmentosum cells, Int. J. Radiat. Biol. 22: 159–165.Google Scholar
  250. MacKee, G. M., and Rosen, I., 1916, Monilethrix. A clinical and histological study, with a report of six cases and a review of the literature. J. Cut. Dis. 34: 444.Google Scholar
  251. Maddox, W. D., Winkelmann, R. K., Harrison, E. G., Devine, K. D., and Gibilisco, J. A., 1964, Multiple nevoid basal cell epitheliomata, jaw cysts and skeletal defects, JAMA. 188: 106–111.CrossRefGoogle Scholar
  252. Malmendier, C. L., Jonniaux, G., Boet, W., and Van Den Bergen, C. J., 1974, Fatty acid composition of tissues in Refsum’s disease. (Heredopathia atactica polyneuritiformis), Biomedicine 20: 398–407.PubMedGoogle Scholar
  253. Malt, R. A., 1965, Keratin in monilethrix, J. Invest. Dermatol. 44: 364.PubMedGoogle Scholar
  254. Marcelo, C. L., 1979, Differential effects of cAMP and cGMP on in vitro epidermal cell growth, Exp. Cell Res. 120: 201–210.PubMedCrossRefGoogle Scholar
  255. Marcelo, C. L., and Duell, E. A., 1979, Cyclic AMP stimulates and inhibits adult human epidermal cell growth, J. Invest. Dermatol. 72: 279.CrossRefGoogle Scholar
  256. Marks, R., 1978, Epidermal activity in the involved and uninvolved skin of patients with psoriasis, Br. J. Dermatol. 98: 399–404.PubMedCrossRefGoogle Scholar
  257. Martin, G. I., Kaiserman, D., Liegler, D., Amorosi, E. D., and Nadel, H., 1976, Computer-assisted cranial tomography in early diagnosis of tuberous sclerosis, JAMA 235: 2323–2324.PubMedCrossRefGoogle Scholar
  258. Mason, J. K., Helwig, E. B., and Graham, J. H., 1965, Pathology of the nevoid basal cell carcinoma syndrome, Arch. Path. 79: 401–408.PubMedGoogle Scholar
  259. Matoltsy, A. G., 1965, Soluble prekeratin, in: Biology of Skin and Hair Growth(A. G. Lyne, and B. F. Short, eds.), pp. 291–305, Angus and Robertson, Sidney.Google Scholar
  260. Matoltsy, A. G., 1977, The membrane of horny cells, in: Biochemistry of Cutaneous Epidermal Differentiation (M. Seiji and I. A. Bernstein, eds.), pp. 93–108, University of Tokyo Press, Tokyo.Google Scholar
  261. Matoltsy, A. G., and Matoltsy, M. N., 1966, The membrane protein of homy cells, J. Invest. Dermatol. 46: 127–129.PubMedGoogle Scholar
  262. Matoltsy, A. G., and Matoltsy, M. N., 1970, Chemical nature of keratohyalin granules of the epidermis, J. Cell Biol. 47: 593–603.PubMedCrossRefGoogle Scholar
  263. Matoltsy, A. G., and Parakkal, P. F., 1965, Membrane-coating granules of keratinizing epithelia, J. Cell Biol. 24: 297–307.PubMedCrossRefGoogle Scholar
  264. Matoltsy, A. G., and Parakkal, P. F., 1967, in: Ultrastructure of Normal and Abnormal Skin, (A. S. Zelickson, ed.), pp. 76–104, Lea and Febiger, Philadelphia.Google Scholar
  265. McEvoy, B. F., and Gatzek, H., 1969, Multiple nevoid basal cell carcinoma syndrome. Radiologic manifestations, Br. J. Radiol. 42: 24–28.PubMedCrossRefGoogle Scholar
  266. Menkes, J. H., Alter, M., Steigleder, G. K., Weakley, D. R., and Sung, J. H., 1962, A sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration, Pediatr. 29: 764–779.Google Scholar
  267. Meyer, J. Ch., Weiss, H., Grundmann, H. P., Wursch, T. G., and Schnyder, U. W., 1979, Deficiency of arylsulfatase C in cultured skin fibroblasts of X-linked ichthyosis, Hum. Genet. 53: 115–116.PubMedCrossRefGoogle Scholar
  268. Mohandas, T., Shapiro, L. J., Sparkes, R. S., and Sparkes, M. C., 1979, Regional assignment of the steroid sulfatase-X-linked ichthyosis locus: Implications for a non-inactivated region on the short arm of human X chromosome, Proc. Natl. Acad. Sci. USA 76(11): 5779–5783.PubMedCrossRefGoogle Scholar
  269. Moll, J. M. H., and Wright, V., 1973, Psoriatic arthritis, Semin. Arthritis Rheum. 3: 55–78.PubMedCrossRefGoogle Scholar
  270. Moshell, A. N., Andrews, A. D., Barrett, S. F., Ganges, M. B., and Robbins, J. G., 1978, Postultraviolet colony-forming ability of fibroblasts from xeroderma pigmentosum patients with only a few neurological abnormalities, Clin. Res. 26: 574A.Google Scholar
  271. Moynahan, E. J., 1974, Acrodermatitis enteropathica: a lethal inherited human zinc-deficiency disorder, Lancet 2: 399–400.PubMedCrossRefGoogle Scholar
  272. Moynahan, E. J., and Grupper, C., 1979, Acrodermatitis enteropathica and other zinc-deficiency disorders, in: Dermatology in General Medicine, 2nd ed. (T. B. Fitzpatrick, A. Z. Eisen, K. Wolff, I. M. Freedberg, and K. F. Austen, eds.), pp. 1371–1375, McGraw-Hill, New York.Google Scholar
  273. Müller, C. R., Migl, R., Traupe, H., and Ropers, H. H., 1980a, X-linked steroid sulfatase: Evidence for different gene-dosage in males and females, Hum. Genet. 54: 197–199.PubMedCrossRefGoogle Scholar
  274. Müller, C. R., Westerveld, A., Migl, B., Franke, W., and Ropers, H. H., 1980b, Regional assignment of the gene locus for steroid sufatase, Hum. Genet. 54: 201–204.PubMedCrossRefGoogle Scholar
  275. Muller, S. A., 1973, Alopecia: Syndromes of genetic significance, J. Invest. Dermatol. 60: 475–491.PubMedCrossRefGoogle Scholar
  276. Muller, S. A., Belcher, R. W., Esterly, N. B., Lochner, J. C., Miller, J. S., Roenigk, H., and Wiesman, L., 1977, Keratinizing dermatoses: combined data from 4 centers on shortterm topical treatment with tretinoin, Arch. Dermatol. 113: 1052–1054.PubMedCrossRefGoogle Scholar
  277. Murphy, J. V., Wolfe, H. J., Balasz, E. A., and Moser, H. W., 1971, A patient with deficiency of arylsulfatase A, B, C and steroid sulfatase, associated with storage of sulfatide, cholesterol sulfate and glycosaminoglycans, in: Lipid Storage Diseases: Enzymatic Defects and Clinical Implications (J. Bernsohn, and H. J. Grossman, eds.), pp. 67–110, Academic Press, New York.Google Scholar
  278. Murphy, J. V., D’Souza, B. J., and Haughton, V. M., 1976, CT scans and tuberous sclerosis, JAMA 236: 1115.PubMedCrossRefGoogle Scholar
  279. Murray, C., Mann, D. L., Gerber, L. N., Barth, W., Perlmann, S., and Decker, J. L., 1980, Histocompatability alloantigens in psoriasis and psoriatic arthritis, J. Clin. Invest. 66: 670–675.PubMedCrossRefGoogle Scholar
  280. Neldner, K. H., and Hambidge, K. M., 1975, Zinc therapy in acrodermatitis enteropathica, New Eng. J. Med. 292: 879–882.PubMedCrossRefGoogle Scholar
  281. Neldner, K. H., Hambidge, K. M., and Walravens, P. A., 1978, Acrodermatitis enteropathica, Int. J. Dermatol. 5(17): 380–387.Google Scholar
  282. Nickel, W. R., and Reed, W. B., 1962, Tuberous Sclerosis, Arch. Dermatol. 85: 209–226.PubMedCrossRefGoogle Scholar
  283. Nicotra, A., 1929, La Calcificazione e l’osteoma della falce del cervello, al controllo anatomo-radiologico e clinico, Arch. Radiol. (Napoli) 5: 794–823.Google Scholar
  284. Nomland, R., 1932, Multiple basal cell epitheliomas originating from congenital pigmented basal cell nevi, Arch. Dermatol. Syph. (Chic.) 25: 1002–1008.CrossRefGoogle Scholar
  285. Oakes, B. W., Danks, D. M., and Campbell, P. E., 1976, Human copper deficiency: Ultrastructural studies of the aorta and skin in a child with Menkes’ syndrome, Expr. Mol. Pathol. 25: 82–98.CrossRefGoogle Scholar
  286. Odland, G. F., 1979, in: Dermatology in General Medicine, 2nd ed., T. B. Fitzpatrick et al., eds., McGraw-Hill, New York.Google Scholar
  287. Orfanos, C. E., ed., 1979, Haar und Haarkrankheiten, Gustav Fischer Verlag, Stuttgart.Google Scholar
  288. Orfanos, C. E., 1980, Oral retinoids—present status. Br. J. Dermatol. 103: 473–480.PubMedCrossRefGoogle Scholar
  289. Orth, G., Favre, M., and Croissant, O., 1977, Characterization of a new type of human papilloma virus that causes skin warts, J. Virol. 24: 108–120.PubMedGoogle Scholar
  290. Orth, G., Jablonska, S., Favre, M., Croissant, O., Jarzabek-Chorzelska, M., and Rzesa, G., 1978, Characterization of two types of human papillomaviruses in lesions of epidermodysplasia verruciformis, Proc. Natl Acad. Sci. USA 75(3): 1537–1541.PubMedCrossRefGoogle Scholar
  291. Orth, G., Jablonska, S., Jarzabek-Chorzelska, M., Obalek, S., Rzesa, G., Favre, M., and Croissant, O., 1979, Characteristics of the lesions and risk of malignant conversion associated with the type of human papillomavirus involved in epidermodysplasia verruciformis, Cancer Res. 39: 1074–1082.PubMedGoogle Scholar
  292. Painter, R. B., and Cleaver, J. E., 1969, Repair replication, unscheduled DNA synthesis, and the repair of mammalian DNARadiat. Res. 37: 451–466.PubMedCrossRefGoogle Scholar
  293. Parakkal, P. F., 1979, Katagen- und telogenphase, in: Haar und Haarkrankheiten, (C. E. Orfanos, ed.), pp. 77–93, Gustav Fischer Verlag, Stuttgart.Google Scholar
  294. Parrish, J. A., Baden, H. P., Goldsmith, L. A., and Matz, M. H., 1972, Studies of the density and the properties of the hair in a new inherited syndrome of hypotrichosis, Ann. Hum. Genet. London 35: 349–356.CrossRefGoogle Scholar
  295. Parsons, W. B., 1961, Treatment of hypercholesteremia by nicotinic acid, Arch. Int. Med. 107: 639–652.CrossRefGoogle Scholar
  296. Pasini, A., 1928, Dystrophie cutanee bulleuse atrophiante et albopapuloide, Ann. Dermatol. Syph. (Paris) 9: 1044–1066.Google Scholar
  297. Pass, F., 1974, Warts: Biology and current therapy, Minn. Med. 57: 844–847.PubMedGoogle Scholar
  298. Patterson, M. C., Lohman, P. H., and Sluyter, M. L., 1973, Use of a UV-endonuclease from Micrococcus luteus to monitor the progress of DNA repair in UV-irradiated human cells, Mutat. Res. 19: 245–256.CrossRefGoogle Scholar
  299. Pearson, R. W., 1971, The mechanobullous disease (epidermolysis bullosa), in Dermatology in General Medicine (T. B. Fitzpatrick, K. A. Arndt, W. H. Clark, Jr., A. Z. Eisen, E. J. Van Scott, and J. H. Vaughan, eds.), pp. 621–643, McGraw-Hill, New York.Google Scholar
  300. Pearson, R. W., Potter, B., and Strauss, F., 1974, Epidermolysis bullosa hereditaria letalis. Clinical and histological manifestations and course of the disease, Arch. Dermatol. 109: 349–355.PubMedCrossRefGoogle Scholar
  301. Pfister, H., and zur Hausen, H., 1978, Seroepidemiological studies of human papilloma virus (HPB-1) infections, Int. J. Cancer 21: 161: 165.Google Scholar
  302. Philipsen, H. P., 1956, Om Keratocyster (kolesteatomer) i kaeberne, Tandleagebladet 60: 963.Google Scholar
  303. Pollitt, R. J., Jenner, F. A., and Davies, M., 1968, Sibs with mental and physical retardation and trichorrhexis nodosa with abnormal amino acid composition of hair, Arch. Dis. Child. 43: 211–216.PubMedCrossRefGoogle Scholar
  304. Pope, F. M., Rees, J. K., Wells, R. S., and Lewis, K. G., 1972, Outpatient treatment of ichthyosis: A double-blind trial of ointments, Br. J. Dermatol. 86: 291–296.PubMedCrossRefGoogle Scholar
  305. Porter, P. S., 1973, Genetic disorders of hair growth, J. Invest. Dermatol. 60: 493–502.PubMedCrossRefGoogle Scholar
  306. Potter, J. L., Timmons, G. D., West, R., and Silvidi, A. A., 1974, Argininosuccinicaciduria, Am. J. Dis. Child. 127: 724–727.PubMedGoogle Scholar
  307. Prawer, S. E., Pass, F., Vance, J. C., Greenberg, L. J., Yunis, E. J., and Zelickson, A. S., 1977, Depressed immune function in epidermodysplasia verruciformis, Arch. Dermatol. 113: 495–497.PubMedCrossRefGoogle Scholar
  308. Press, N., Kikuchi, H., Shimoyama, T., and Thompson, G. R., 1974, Diagnosis and treatment of essential fatty deficiency in man, Brit. Med. J. 2: 247–250.PubMedCrossRefGoogle Scholar
  309. Price, V., in press, Trichothiodystrophy, sulfur deficient brittle hair, in: Hair, Trace Elements and Human Illness (A.C. Brown and R. G. Crounse, eds.), Praeger Publishers, New York.Google Scholar
  310. Proctor, M., Fletcher, H., Shukla, J., and Rennert, O., 1975, Elevated spermidine and spermine levels in the blood of psoriasis patients, J. Invest. Dermatol. 65: 409–411.PubMedCrossRefGoogle Scholar
  311. Prystowsky, S. D., Herndon, J. H., Freeman, R. G., and Boudreau, C., 1976, Epidermodysplasia verruciformis, Am. J. Dis. Child. 130: 439–440.Google Scholar
  312. Pyrohonen, S., Jablonska, S., Obalek, S., and Kuismanen, E., 1980, Immune reactions in epidermodysplasia verruciformis. Br. J. Dermatol. 102: 247–254.CrossRefGoogle Scholar
  313. Rabson, A. S., Tyrrell, S. A., and Legallais, F. Y., 1969, Growth of ultraviolet-damaged herpesvirus in xeroderma pigmentosum cells, Proc. Soc. Exp. Bull. Med. 132: 802–806.Google Scholar
  314. Race, R. R., and Sanger, R., 1975, Blood Groups in Man, Chapters 28–30, pp. 578–630, Blackwell, London.Google Scholar
  315. Racy, A., Barakat, A. Y., and Cochran, W. E., 1978, Computerized axial tomography in family members of patients with tuberous sclerosis, Clin. Pediatr. 17: 883–885.CrossRefGoogle Scholar
  316. Rajagopalan, K., Bahry, J., Tay, C. H., Chin, K. N., and Tan, K. K., 1972, Familial epidermodysplasia verruciformis of Lewandowsky and Lutz, Arch. Dermatol. 102: 73–78.CrossRefGoogle Scholar
  317. Rasmussen, P. E., 1963, Follikulaere kaebecyster, basal-celle-tumorer og knogleanomalier som led i et hereditareret syndrom, Nord. Med. 69: 606–613.PubMedGoogle Scholar
  318. Reed, W. B., Herwick, R. P., Harville, D., Porter, P. S., and Conant, N., 1972, Lamellar ichthyosis of the newborn. A distinct clinical entity; its comparison to other ichthyosiform erythrodermas, Arch. Dermatol. 105: 394–399.PubMedCrossRefGoogle Scholar
  319. Regan, J. D., and Setlow, R. B., 1976, Repair of human DNA: Radiation and chemical damage in normal and xeroderma pigmentosum cells, in: Biology of Radiation Carcinogenesis (J. M. Yuhas, R. W. Tennant, and J. D. Regan, eds.), pp. 103–113, Raven Press, New York.Google Scholar
  320. Riccardi, V. M., 1977, Cellular interaction as a limiting factor in the expression of oncogenic mutations: An hypothesis, in: Genetics of Human Cancer (J. Mulvihill, R. W. Miller, and J. F. Fraumeni, Jr., eds.), pp. 383–385, Raven Press, New York.Google Scholar
  321. Riccardi, V. M., 1979, Cell-cell interaction as an epigenetic determinant in the expression of mutant neural crest cells, Birth Defects 15(8): 89–98.PubMedGoogle Scholar
  322. Riccardi, V. M., 1980, Pathophysiology of neurofibromatosis. IV. Dermatologic insights into heterogeneity and pathogenesis, J. Am. Acad. Dermatol. 3: 157–166.PubMedCrossRefGoogle Scholar
  323. Riccardi, V. M., and Maragos, V. A., 1980, The pathophysiology of neurofibromatosis. I. Resistance in Vitro to 3-Nitrotyrosine as an expression of the mutation, In Vitro 16(8): 706–714.PubMedCrossRefGoogle Scholar
  324. Rice, R. H., and Green, H., 1977, The cornified envelope of terminally differentiated human epidermal keratinocytes consist of cross-linked protein, Cell 11: 417–422.PubMedCrossRefGoogle Scholar
  325. Rice, R. H., and Green, H., 1979, Presence in human epidermal cells of a soluble protein precursor of the cross-linking by calcium ions, Cell 18: 681–694.PubMedCrossRefGoogle Scholar
  326. Richner, H., 1938, Hornhautaffektion bei keratoma palmare et plantare heriditarium, Klin. Monatsbl. Augenheilkd. 100: 580–588.Google Scholar
  327. Robbins, J. H., 1978, Significance of repair of human DNA: Evidence from studies of xeroderma pigmentosum, J. Nat. Cancer Inst. 61(3): 645–656.PubMedGoogle Scholar
  328. Robbins, J. H., 1979, Xeroderma pigmentosum, in: Dermatology in General Medicine, 2nd ed. (T. B. Fitzpatrick, A. Z. Eisen, K. Wolff, I. M. Freedberg, and K. F. Austen, eds.), pp. 390–394, McGraw-Hill, New York.Google Scholar
  329. Robbins, J. H., Kraemer, K. H., Lutzner, M. A., Festoff, B. W., and Coon, H. G., 1974, Xeroderma pigmentosum: an inherited disease with sun sensitivity, multiple cutaneous neoplasms, and abnormal DNA repair, Ann. Intern. Med. 80: 221–248.PubMedGoogle Scholar
  330. Robbins, J. H., Andrews, A. D., and Moshell, A. N., 1978a, DNA repair nucleotide sequences which prevent premature death of neurons in humans: Evidence from studies on xeroderma pigmentosum, in: DNA Repair Mechanisms (P. C. Hanawalt, E. C. Friedberg, and C. F. Fox, eds.), pp. 609–612, Academic Press, New York.Google Scholar
  331. Robbins, J. H., Andrews, A. D., Moshell, A. N., 1978b, Evidence that DNA repair processes protect normal humans from premature solar skin damage and from premature neuronal death, J. Supramol. Struct. (Suppl.) 2: 28.Google Scholar
  332. Robertson, A. F., Schuerger, G. S., Brown, R. R., and Karp, W. B., 1973, Tryptophan metabolism in acrodermatitis enteropathica, J. Pediatr. 83: 1012–1016.PubMedCrossRefGoogle Scholar
  333. Rochman, H., Cooper, M., Esterly, N. B., and Bauer, E. A., 1979, Carcinoembryonic antigen: Increased plasma levels in recessive epidermolysis bullosa, J. Invest. Dermatol. 72: 262–263.PubMedCrossRefGoogle Scholar
  334. Rogers, G.E., 1964, Structural and biochemical features of the hair follicle, in: The Epidermis (W. Montagna and W. C. Lobitz, eds.), p. 179, Academic Press, New York.Google Scholar
  335. Rogers, G. E., and Simmonds, D. H., 1958, Content of citrulline and other amino acids in a protein of hair follicles, Nature 182: 186–187.PubMedCrossRefGoogle Scholar
  336. Rook, A., Wilkinson, D. S., and Ebling, F. J. G. (eds.), 1972, Refsum’s syndrome, in: Textbook of Dermatology, 2nd ed., p. 158, Blackwell, Oxford.Google Scholar
  337. Roth, S. I., and Clark, W. H., 1964, Ultrastructural evidence related to the mechanism of keratin synthesis, in: The Epidermis (W. Montagna, and W. C. Lobitz, eds.), Academic Press, New York.Google Scholar
  338. Rothberg, S., Crounse, R. G., and Lee, J. L., 1961, Glycine-C14 incorporation into the proteins of normal stratum corneum and abnormal stratum corneum of psoriasis, J. Invest. Dermatol. 37: 497–505.PubMedCrossRefGoogle Scholar
  339. Rowson, K., and Mahy, B., 1967, Human papova (wart) virus, Bacteriol. Rev. 31: 110–131.PubMedGoogle Scholar
  340. Rudall, K. M., 1952, The protein of mammalian epidermis, in: Advances in Protein Chemistry, Volume 7 (M. L. Anson, K. Bailey, and J. T. Edsall, eds.), pp. 253–290, Academic Press, New York.Google Scholar
  341. Ruiter, M., 1969, Malignant degeneration of skin lesions in epidermodysplasia verruciformis, Acta Derm. Venereol. 49: 309–313.PubMedGoogle Scholar
  342. Ruokonen, A., Oikarinen, A., Palatsi, R., and Huhtaniemi, I., 1980, Serum steroid sulphates in ichthyosis, Br. J. Dermatol. 102: 245–248.CrossRefGoogle Scholar
  343. Russel, J. T., Schultes, M. L., and Kuban, D. J., 1972, Histocompatibility (HL-A) antigens associated with psoriasis, New Eng. J. Med. 287: 738–740.CrossRefGoogle Scholar
  344. Russell, D. H., Combest, W. L., Duell, E. A., Stawiski, M. A., Anderson, T. F., and Voorhees, J. J., 1978, Glucocorticoid inhibits elevated polyamine biosynthesis in psoriasis, J. Invest. Dermatol. 71: 177–181.PubMedCrossRefGoogle Scholar
  345. Ryan, T. J., 1980, Microcirculation in psoriasis: blood vessels, lymphatics and tissue fluid, Pharm. Ther. 10: 27–64.CrossRefGoogle Scholar
  346. Sachs, G., and Braun-Falco, O., 1960, The occurrence and nature of arylsulfatases in parakeratosis, J. Invest. Dermatol. 34: 439–444.PubMedCrossRefGoogle Scholar
  347. Salamon, T., and Schnyder, U. W., 1962, On monilethrix, Arch. Klin. Exp. Dermatol. 215: 105–136.PubMedCrossRefGoogle Scholar
  348. Sanger, R., Tipett, P., Gavin, J., Teesdale, P., and Daniels, G. L., 1977, Xg groups and sex chromosome abnormalities in people of northern european ancestry, J. Med. Genet. 39: 277–281.Google Scholar
  349. Sasai, Y., Saito, N., and Seiji, M., 1973, Epidermolysis bullosa dystrophica et albo-papuloidea, Arch. Dermatol. 108: 554–557.PubMedCrossRefGoogle Scholar
  350. Sass-Kortsak, A., and Bearn, A. G., 1978, Menkes’ disease (kinky-hair or steely-hair syndrome), in: The Metabolic Basis of Inherited Disease, 4th ed. (J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, eds.), pp. 1116–1119, McGraw-Hill, New York.Google Scholar
  351. Savolainen, E. R., Kero, M., Pihlajaniemi, T., and Kivirikko, K. I., 1981, Deficiency of galactosylhydroxylysyl glucosyltransferase, and enzyme of collagen synthesis, in a family with dominant epidermolysis bullosa simplex, New Eng. J. Med. 303(4): 197–204.CrossRefGoogle Scholar
  352. Schwartz, T. M., and Shackelford, R. M., 1973, Pseudodistemper is apparently new ailment of mink, U.S. Fur Rancher 52: 6.Google Scholar
  353. Scriver, C. R., and Rosenberg, L. E., 1973, Amino acid metabolism and its disorders, Major Probl. Clin Pediatr. 10: 338–352.Google Scholar
  354. Segal, S., 1978, Disorders of galactose metabolism, in: The Metabolic Basis of Inherited Disease, 4th ed. (J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, eds.), pp. 160–182, McGraw-Hill, New York.Google Scholar
  355. Shapiro, L., 1979, Steroid sulfatase deficiency and X-linked ichthyosis, Clin. Biochem. 12: 205.PubMedCrossRefGoogle Scholar
  356. Shapiro, L. J., Cousins, L., Fluharty, A. L., Stevens, R. L., and Kihara, H., 1977, Steroid sulfatase deficiency, Pediatr. Res. 11: 894–897.PubMedGoogle Scholar
  357. Shapiro, L. J., Weiss, R., Webster, D., and France, J. T., 1978a, X-linked ichthyosis due to steroid-sulphatase deficiency, Lancet 1: 70–72.PubMedCrossRefGoogle Scholar
  358. Shapiro, L. J., Weiss, R., Buxman, M. M., Vidgoff, J., Dimond, R. L., Roller, J. A., and Wells, R. S., 1978b, Enzymatic basis of typical X-linked ichthyosis, Lancet 8093: 756–757.CrossRefGoogle Scholar
  359. Shear, M., 1960, Primordial cysts, J. Dent. Assoc. S. Afr. 15: 211.Google Scholar
  360. Shechter, Y., Landau, J. W., and Newcomer, V. D., 1969, Comparative disc electrophoresis of hair keratins, J. Invest. Dermatol. 52: 57–62.PubMedGoogle Scholar
  361. Shelley, W. B., and Rawnsley, H. M., 1965, Aminogenic alopecia loss of hair associated with argininosuccinic aciduria, Lancet 2: 1327–1328.CrossRefGoogle Scholar
  362. Shih, V. E., 1978, Argininosuccinase deficiency (Argininosuccinic aciduria), in: The Metabolic Basis of Inherited Disease, 4th ed. (J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, eds.), pp. 373–386, McGraw-Hill, New York.Google Scholar
  363. Shih, V. E., Littlefield, J. W., and Moser, H. W., 1969, Argininosuccinase deficiency in fibroblasts cultured from patients with argininosuccinic aciduria, Biochem. Genet. 3: 81–83.CrossRefGoogle Scholar
  364. Sivasubramanian, K. N., and Henkin, R., 1978, Behavioral and dermatologic changes and low serum zinc and copper concentrations in two premature infants after parenteral alimentation, J. Pediatr. 93(5): 847–851.PubMedCrossRefGoogle Scholar
  365. Skerrow, D., and Hunter, I., 1978, Protein modifications during the keratinization of normal and psoriatic human epidermis, Biochim. Biophys. Acta 537: 474–484.PubMedGoogle Scholar
  366. Skerrow, C. J., and Matoltsy, A. G., 1974a, Isolation of epidermal desmosomes, J. Cell Biol. 63: 515–523.PubMedCrossRefGoogle Scholar
  367. Skerrow, C. J., and Matoltsy, A. G., 1974b, Chemical characterization of isolated epidermal desmosomes, J. Cell Biol. 63: 524–530.PubMedCrossRefGoogle Scholar
  368. Smith, K. C., 1977, in: The Science of Photobiology, K. C. Smith, ed., Plenum, New York.Google Scholar
  369. Solomon, I. L., and Green, O. C., 1963, Monilethrix. Its occurrence in seven generations with one case that responded to endocrine therapy, New Eng. J. Med. 269: 1279–1282.PubMedCrossRefGoogle Scholar
  370. Steinberg, G., Avigan, J., Mize, C., Eldjarn, L., Try, K., and Refsum, S., 1965, Conversion of U-C14 phytol to phytanic acid and its oxidation in heredopathia atactica polyneuritiformis, Biochem. Biophys. Res. Commun. 19: 783.PubMedCrossRefGoogle Scholar
  371. Steinert, P. M., 1977, The mechanism of assembly of bovine epidermal keratin filaments in vitro, in: Biochemistry of Cutaneous Epidermal Differentiation (M. Seiji, and I. A. Bernstein, eds., pp. 444–466, University of Tokyo Press, Tokyo.Google Scholar
  372. Steinert, P. M., and Idler, W. W., 1979, Post synthetic modifications of mammalian epidermal α-keratin. Biochemistry 18: 5664–5669.PubMedCrossRefGoogle Scholar
  373. Straith, F. E., 1939, Hereditary epidermoid cyst of jaws, Am. J. Orthodont. 25: 673–691.CrossRefGoogle Scholar
  374. Sugawara, K., 1977, Intermolecular cross-links in epidermal differentiation, in: Biochemistry of Cutaneous Epidermal Differentiation (M. Seiji, and I. A. Bernstein, eds.), pp. 387–397, University of Tokyo Press, Tokyo.Google Scholar
  375. Summerly, R., and Hale, A. J., 1965, Basal cell naevus syndrome, Trans. St. Johns Hosp. Derm. Soc. (Lond.) 51: 77–79.Google Scholar
  376. Swanbeck, G. A., 1968, A new treatment of ichthyosis and other hyperkeratotic conditions, Acta Dermato-Venereol. 48: 123–127.Google Scholar
  377. Tasman-Jones, C., 1980, Zinc deficiency states, in: Advances in Internal Medicine, Volume 26 (G. H. Stollerman, W. J. Harrington, J. B. Kirsner, C. E. Kossmann, and M. D. Siperstein, eds.), pp. 97–114, Year Book Medical Publishers, Chicago, Illinois.Google Scholar
  378. Taylor, W. B., Anderson, D. E., Howell, J. B., and Thurston, C. S., 1968, The nevoid basal cell carcinoma syndrome, Arch. Dermatol. 98: 612–614.PubMedCrossRefGoogle Scholar
  379. Thaler, M. P., Fukuyama, K., Inoue, N., Cram, D. L., and Epstein, W. L., 1978, 2-Tris urea mercaptoethanol extractable polypeptides found uniquely in scales of patients with psoriasis, J. Invest. Dermatol. 70: 38–41.PubMedCrossRefGoogle Scholar
  380. Thaler, M., Fukuyama, K., Epstein, W. L., and Fisher, K. A., 1980, Comparative studies of keratins from psoriasis and atopic dermatitis, J. Invest. Dermatol. 75: 156–158.PubMedCrossRefGoogle Scholar
  381. Thivolet, J., Hegazy, M., Viac, J., and Chardonnet, V., 1977, An in vivo study of cell- mediated immunity in human warts, Acta Derm. Venereol (Stockh.) 57: 317–319.Google Scholar
  382. Thune, P., 1980, A case of centrolobular toxic necrosis of the liver due to aromatic retinoid— tigason (RO-10–9359), Dermatologica 160: 405–408.PubMedCrossRefGoogle Scholar
  383. Tiepolo, I., Zuffardi, O., and Rodewald, A., 1977, Nullisomy for the distal portion of Xp in a male child with Xly translocation, Hum. Genet. 39: 277–281.PubMedCrossRefGoogle Scholar
  384. Tiepolo, L., Zuffardi, O., Fraccaro, M., diNatale, D., Gargantini, L., Muller, C. R., and Ropers, H. H., 1980, Assignment by deletion mapping of the steroid sulfatase X-linked ichthyosis locus to Xp223, Hum. Genet. 54: 205–206.PubMedCrossRefGoogle Scholar
  385. Tomlinson, S., and Westall, R. G., 1964, Argininosuccinic aciduria, argininosuccinase and arginase in human blood cells, Clin. Sci. 26: 261–269.PubMedGoogle Scholar
  386. Touraine, M. A., 1942, Classification des epidermolyses bulleuses, Ann. Dermatol. Syph. (Paris) 2: 141–144.Google Scholar
  387. Trosko, J. E., Krause, D., and Isoun, M., 1970, Sunlight-induced pyrimidine dimers in human cells in vitro, Nature 228: 358–359.PubMedCrossRefGoogle Scholar
  388. Valle, K.-J., and Bauer, E. A., 1980, Enhanced biosynthesis of human skin collagenase in fibroblast cultures from recessive dystrophic epidermolysis bullosa, J. Clin. Invest. 66: 176–187.PubMedCrossRefGoogle Scholar
  389. Vandersteen, P. R., and Muller, S. A., 1972, Lamellar ichthyosis. An enzyme, histochemical, light and electron microscopic study, Arch. Dermatol. 106: 694–701.PubMedCrossRefGoogle Scholar
  390. Van Scott, E. J., and Yee, R. J., 1974, Control of keratinization with α-hydroxy acid and related compounds. I. Topical treatment of ichthyotic disorders, Arch. Dermatol. 110: 586–590.PubMedCrossRefGoogle Scholar
  391. Verbov, J., and McCarthy, K., 1978, Accidental vaccinia in an atopic and her ichthyotic sister, Lancet 8069: 870.CrossRefGoogle Scholar
  392. Verbov, J., Munro, D. D., and Miller, A., 1972, Recurrent eczema herpeticum associated with ichthyosis vulgaris, Br. J. Dermatol. 86: 638–642.PubMedCrossRefGoogle Scholar
  393. Viac, J., Thivolet, J., Hegazy, M., Chardonnet, Y., and Dambuyant, C., 1977, Comparative study of delayed hypersensitivity skin reactions and antibodies to human papilloma virus (HPV), Clin. Exp. Immunol. 29: 240–246.PubMedGoogle Scholar
  394. Viac, J., Staquet, M. J., Thivolet, J., and Goujon, C., 1980, Experimental production of antibodies against stratum corneum keratin polypeptides, Arch. Dermatol. Res. 267: 179–188.PubMedCrossRefGoogle Scholar
  395. Vihko, R., and Ruokonen, A., 1975, Steroid sulphates in human adult testicular steroid synthesis, J. Steroid Biochem. 6: 353–356.PubMedCrossRefGoogle Scholar
  396. Vogt, H., 1908, Zur pathologic und pathologischen der verscheidenen idiotieformen. II. Tuberöse Sklerose, Monatsschr. Psychol. Neurol. 24: 106–150.CrossRefGoogle Scholar
  397. von Recklinghausen, F. D. 1882, Ueber die multiplen Fibrome der Haut: Ihre Beziehung zu den multiplen Neuromen, Hirschwald, Berlin.Google Scholar
  398. Voorhees, J. J., Duell, E. A., Bass, L. J., Powell, J. A., and Harrell, E. R., 1962, Decreased cyclic AMP in the epidermis of lesions of psoriasis, Arch. Dermatol. 105: 695–701.CrossRefGoogle Scholar
  399. Voorhees, J. J., Kelsey, W., Sawiski, M., Smith, E., Duell, E. A., Haddox, M., and Goldberg, N., 1973, Increased cyclic GMP and decreased cyclic AMP levels in the rapidly proliferating epithelium of psoriasis, in: The Role of Cyclic Nucleotides in Carcinogenesis, Volume 6, Proceedings from the Miami Winter Symposia (J. Schultz and H. G. Gratzner, eds.), Academic Press, New York.Google Scholar
  400. Wadman, S. K., Van Sprang, F. J., Maas, J. W., and Ketting, D., 1968, An exceptional case of tyrosinosis, J. Ment. Defic. Res. 12: 269–281.PubMedGoogle Scholar
  401. Ward, W. H., 1960. Naevoid basal cell carcinoma associated with dyskeratosis of palms and soles: new entity, Aust. J. Dermatol. 5: 204–208.PubMedCrossRefGoogle Scholar
  402. Warshawsky, R. S., Hill, C. W., Doughman, D. J., and Harris, J. E., 1975, Genodermatoses and congenital disorders, Arch. Ophthal. 93: 194–197.PubMedCrossRefGoogle Scholar
  403. Watson, W., Cann, H. M., Farber, E. M., and Nall, M. L., 1972, The genetics of psoriasis, Arch. Dermatol. 105: 197–207.PubMedCrossRefGoogle Scholar
  404. Weinstein, G. D., and Frost, P., 1968, Abnormal cell proliferation in psoriasis, J. Invest. Dermtol. 50: 254–259.Google Scholar
  405. Weinstein, G. D., and Van Scott, E. J., 1965, Autoradiographic analysis of turnover times of normal and psoriatic epidermis, J. Invest. Dermatol. 45: 257–262.PubMedGoogle Scholar
  406. Wells, R. S., and Kerr, C. B., 1966a, The histology of ichthyosis, J. Invest. Dermatol. 46: 530–535.Google Scholar
  407. Wells, R. S., and Kerr, C. B., 1966b, Clinical features of autosomal dominant and sex linked ichthyosis in an English population, Br. Med. J. 1: 947–950.PubMedCrossRefGoogle Scholar
  408. Weston, W. L., Huff, J. C., Humbert, J. R., Hambidge, M., Neldner, K. H., and Walravens, P. A., 1977, Zinc correction of defective chemotaxis in acrodermatitis enteropathica, Arch. Dermatol. 113: 422–425.PubMedCrossRefGoogle Scholar
  409. Wheeler, E. M., and Roberts, P. F., 1976, Menkes’ steely hair syndrome, Arch. Dis. Child. 51: 269–274.PubMedCrossRefGoogle Scholar
  410. White, H. S., Newcomer, V. D., and Terasaki, P. I., 1972, HLA antigens in psoriasis, New Eng. J. Med. 287: 740–743.PubMedCrossRefGoogle Scholar
  411. Wilgram, G. E., and Caulfield, T. B., 1966, An electron microscopic study of epidermolytic hyperkeratoses, Arch. Dermatol. 94: 127–143.PubMedCrossRefGoogle Scholar
  412. Williams, R. S., Marshall, P. C., Lott, I. T., and Coviners, V. S., 1978, The cellular pathology of Menkes steely hair syndrome, Neurology 28: 575–583.PubMedGoogle Scholar
  413. Winkelman, R. K., Perry, H. O., Achor, R. W. P., and Kirby, T. J., 1963, Cutaneous syndromes produced as side effects of triparanol therapy, Arch. Dermatol. 87: 372–377.CrossRefGoogle Scholar
  414. Young, D. F., Eldridge, R. E., Nager, G. T., Deland, F. H., and McNew, J., 1971, Hereditary bilateral acoustic neuroma (central neurofibromatosis), Birth Defects: Original Articles Series 7(4): 73–86.Google Scholar
  415. Zaleski, W. A., Hill, A., and Kushniruk, W., 1973, Skin lesions in tyrosinosis: Response to dietary treatment, Br. J. Dermatol. 88: 335–340.PubMedCrossRefGoogle Scholar
  416. Zelkowitz, M. and Stambouly, J. 1981, Neurofibromatosis fibroblasts: slow growth and abnormal morphology, Pediatr. Res. 15: 290–293.PubMedCrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1982

Authors and Affiliations

  • Howard P. Baden
    • 1
  • Phillip A. Hooker
    • 1
  1. 1.Massachusetts General HospitalHarvard Medical SchoolBostonUSA

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