Epidemiology of Autoimmune Skin Disorders

  • Berthold Rzany
  • Niels Weller


Epidemiology is for most medical doctors a diffuse subject. It is often associated with biostatistics which most doctors believe to be something impossible to understand and besides that assumed to be of little use. Nevertheless every medical textbook has a section on the epidemiology of a specific disease. This section is unfortunately often a trash can of clinical features, risk factors based on case reports and case series. If data of descriptive and analytical epidemiological studies are presented it is often done unenthusiastically. In the book “Dermatologie and Venerologie” by Braun-Falco et al. (1996) for example the section about the epidemiology of a specific disease is hardly longer than two or three sentences and an independent chapter on epidemiological data is completely missing.


Systemic Lupus Erythematosus Systemic Lupus Erythematosus Patient Systemic Sclerosis Connective Tissue Disease Bullous Pemphigoid 


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  1. Asboe-Hansen G (1985) Epidemiology of progressive systemic sclerosis in Denmark. In: Black CM, Myers AR (eds) Systemic Sclerosis (Scleroderma). Gower, New York, p 78Google Scholar
  2. Ball C, Sackett D, Phillips B, Haynes B, Straus S (1999) Levels of evidence and grades of recommendationsGoogle Scholar
  3. Bastuji-Garin S, Joly P, Picard-Dahan C, Bernard P, Vaillant L, Pauwels C, Salagnac V, Lok C, Roujeau JC (1996) Drugs associated with bullous pemphigoid. A case-control study. Arch Dermatol 132: 272–276Google Scholar
  4. Bernard P, Vaillant J, Labeille B, Bedane C, Arbeille B, Denoeux JP et al. (1995) Incidence and distribution of subepidermal bullous skin diseases in three french regions. Arch Dermatol 131: 48–52PubMedCrossRefGoogle Scholar
  5. Braun-Falco O, Plewig G, Wolff HH (1996) Dermatologie und Venerologie, 4th edn Springer, Berlin Heidelberg New YorkGoogle Scholar
  6. Burns CG (1994) The epidemiology of systemic sclerosis: a population-based case-control study, PhD dissertation ( Epidemiological Sciences ), University of MichiganGoogle Scholar
  7. Connell EB (1998) The exploitation of autoimmune disease: breast implant litigation and its dire implications for women’s health. J Womens Health 7: 329–338PubMedCrossRefGoogle Scholar
  8. Edworthy SM, Martin L, Barr SG, Birdsell DC, Brant RF, Fritzler MJ (1998) A clinical study of the relationship between silicone breast implants and connective tissue disease. J Rheumatol 25: 254–260PubMedGoogle Scholar
  9. Englert HJ, Brooks P (1994) Scleroderma and augmentation mammoplasty–a causal relationship? Austr NZ J Med 24: 74–80CrossRefGoogle Scholar
  10. Englert H, Small-McMahon J, Davis K, O’Connor H, Chambers P, Brooks P (1999) Systemic Sclerosis prevalence and mortality in Sydney 1974–88. Aust NZ J Med 29: 42–50CrossRefGoogle Scholar
  11. Fessel WJ (1974) Systemic lupus erythematosus in the community. Incidence, prevalence, outcome, and first symptomes; the high prevalence in black women. Arch Intern Medicin 134: 1027–1035CrossRefGoogle Scholar
  12. Fujikawa S, Okuni M (1997) A nationwide surveillance study of rheumatic diseases among Japanese children. Acta Paediatr Jpn 39: 242–244PubMedCrossRefGoogle Scholar
  13. Gabriel SE, O’Fallon WM, Kurland LT, Beard CM, Woods JE, Melton LJ (1994) Risk of connective-tissue diseases and other disorders after breast implantation. N Engl J Med 330: 1697–1702PubMedCrossRefGoogle Scholar
  14. Gallego H, Crutchfield CE, Lewis EJ, Gallego HJ (1999) Report of an association between discoid lupus erythematosus and smoking. Cutis 63: 231–234PubMedGoogle Scholar
  15. Geirsson AJ, Steinsson K, Guthmundsson S, Sigurthsson (1994) Systemic sclerosis in Iceland: A nationwide epidemiological study. Ann Rheum Dis 53: 502–505Google Scholar
  16. Giltay EJ, Moens HJB, Riley AH, Tan RG (1994) Silicone breast protheses and rheumatic symptoms: a retrospective follow-up study. Ann Rheum Dis 53: 194–196PubMedCrossRefGoogle Scholar
  17. Goldman JA, Greenblatt J, Joines R, White L, Aylward B, Lamm SH (1995) Breast implants, rheumatoid arthritis, and connective tissue diseases in a clinical practice. J Clin Epidemiol 48: 571–582PubMedCrossRefGoogle Scholar
  18. Grattan CEH (1985) Evidence of an association between bullous pemphigoid and psoriasis. Br J Dermatology 113: 281–283CrossRefGoogle Scholar
  19. Guillaume JC, Roujeau JC, Touraine R (1984) Systemic lupus after breast prosthesis. Ann Dermatol Venerol 111: 703–704Google Scholar
  20. Gutierrez FJ, Espinoza LR (1990) Progressive systemic sclerosis complicated by severe hypertension: reversal after silicone implant removal. Am J Med 89: 390–392PubMedCrossRefGoogle Scholar
  21. Haga HJ, Haaland P, Haga T (1992) Silicone breast implants and collagen diseases. Tidsskr Nor Laegeforen 112: 763–764PubMedGoogle Scholar
  22. Hammer M, Krippner H (1991) Three cases of chronic polyarthritis after silicon implantation (breast augmentation): causal realtionship? Immun Infekt 19: 62–63PubMedGoogle Scholar
  23. Hennekens CH, Lee IM, Cook NR, Hebert PR, Karlson EW, LaMotte F, Manson JAE, Buring JE (1996) Self-reported breast implants and connective-tissue diseases in female health professionals–A retrospective cohort study. JAMA 275: 616–621PubMedCrossRefGoogle Scholar
  24. Hochberg MC (1985) The incidence of lupus erythematosus in Baltimore, Maryland 19701977. Arthritis Rheum 28: 80–86PubMedCrossRefGoogle Scholar
  25. Hochberg MC, Perlmutter DL, Medsger TA Jr, Nguyen K, Steen V, Weisman MH, White B, Wigley FM (1996) Lack of association between augmentation mammoplasty and systemic sclerosis (scleroderma). Arthritis Rheum 39: 1125–1131PubMedCrossRefGoogle Scholar
  26. Hopkinson ND, Doherty M, Powell RJ (1993) The prevalence and incidence of systemic lupus erythematosus in Nottingham, UK, 1989–1990. Br J Rheumatol 32: 110–115PubMedCrossRefGoogle Scholar
  27. Hopkinson ND, Doherty M, Powell RJ (1994) Clinical features and race-specific incidence/ prevalence rates of systemic lupus erythematosus in a geographically complete cohort of patients. Ann Rheum Dis 53: 675–680PubMedCrossRefGoogle Scholar
  28. Hopkinson ND, Jenkinson C, Muir KR, Doherty M, Powell RJ (2000) Racial group, socioeconomic status, and the development of persistent proteinuria in systemic lupus erythematosus. Ann Rheum Dis 59: 116–119PubMedCrossRefGoogle Scholar
  29. Jick H, Jick SS, Derby LE (1991) Validation of information recorded on general practitioner based computerised data resource in the United Kingdom. BMJ 302: 766–768PubMedCrossRefGoogle Scholar
  30. Johnson AE, Cavalcanti FS, Gordon C, Nived O, Palmer RG, Sturfelt G, Viner NJ, Bacon PA (1994) Cross-sectional analysis of the differences between patients with systemic lupus erythematosus in England, Brazil and Sweden. Lupus 3: 501–506Google Scholar
  31. Jung M, Kippes W, Messer G, Zillikens D, Rzany B (1999) Increased risk of bullous pemphigoid in male and very old patients. A population-based study on incidence. J Am Acad Dermatol 41: 266–268Google Scholar
  32. Kaipiainen-Seppänen O, Aho K (1996) Incidence of rare systemic rheumatic and connective tissue diseases in Finland. J Intern MedGoogle Scholar
  33. Kaipiainen-Seppänen O, Savolainen A (1996) Incidence of chronic juvenile rheumatic diseases in Finland during 1980–1990. Clin Exp Rheumatol 14: 441–444PubMedGoogle Scholar
  34. Karlson EW, Daltroy LH, Lew RA, Wright EA, Partridge AJ, Roberts WN, Stern SH, Straaton KV, Wacholtz MC, Grosflam JM et al. (1995) The independence and stability of socioeconomic predictors of morbidity in systemic lupus erythematosus. Arthritis Rheum 38: 267273Google Scholar
  35. Karlson EW, Daltroy LH, Lew RA, Wright EA, Partridge AJ, Fessel AH, Roberts WN, Stern SH, Straatom KV, Wachotz MC, Kavanaugh AF, Grosflam JM, Liang MH (1997) The relationship of socioeconomic status, race, and modifiable risk factors to outcomes in patients with systemic lupus erythematosus. Arthritis Rheum 40: 47–56PubMedCrossRefGoogle Scholar
  36. Kind P, Goerz G (1987) Klinik and Differentialdiagnose des kutanen Lupus erythematodes. Z Hautkr 62: 1337–1347PubMedGoogle Scholar
  37. Kumagai Y, Shiokawa Y, Medsger TJ, Rodnan GP (1984) Clinical spectrum of CTD after cosmetic surgery. Observations on eighteen patients and review of the Japanese literature. Arthritis Rheum 27: 1–12PubMedCrossRefGoogle Scholar
  38. Lawrence JS, Martins CL, Drake GL (1987) A family survey of lupus erythematosus. I. Heritability. J Rheumatol 14: 913–921PubMedGoogle Scholar
  39. Lilienfeld DE, Stolley PD (1994) Foundations of Epidemiology 3r° edn Oxford University Press, New York OxfordGoogle Scholar
  40. Maricq HR, Weinrich MC, Keil JE, Smith EA, Harper FE, Nussbaum AI, LeRoy EC, McGregor AR, Diat F, Rosal EJ (1989) Prevalence of scleroderma spectrum disorders in the general population of South Carolina. Arthritis Rheum 32: 998–1002PubMedCrossRefGoogle Scholar
  41. Marik PE, Kark AL, Zambakides A (1990) Scleroderma after silicone augmentation mammoplasty. A report of 2 cases. S Afr Med J 77: 212–213PubMedGoogle Scholar
  42. Masouyé I, Schmied E, Didierjean I (1998) Bullous pemphigoid and multiple sclerosis: more than a coincidence? J Am Acad Dermatol 21: 63–68CrossRefGoogle Scholar
  43. Mayes MD, Laing TJ, Gillespie BW et al. (1996) Prevalence, incidence and survival rates of systemic sclerosis in the Detroit metropolitan area. Arthritis Rheum 39: 5150Google Scholar
  44. Meier CR, Sturkenboom MCJM, Cohen AS, Jick H (1998) Postmenopausal Estrogen Replacement Therapy and the Risk of Developin Systemic Lupus Erythematosus or Discoid Lupus. J Rheumatol 25: 1515–1519PubMedGoogle Scholar
  45. Michet CJ Jr, McKenna CH, Elveback LR, Kaslow RA, Kurland LT (1985) Epidemiology of systemic lupus erythematosus and other connective tissue diseases in Rochester, Minnesota,1950 through 1979. Mayo Clin Proc 60: 105–113PubMedCrossRefGoogle Scholar
  46. Miyoshi K, Miyaoka T, Kobayashi Y, Itakura T, Nishijo K, Higashibara M, Shiragami H, Ohno F (1964) Hypergammaglobulinemia by prolonged adjuvanticity in man: disorders developed after augmentation mammoplasty. Ijishimpo 2122: 9Google Scholar
  47. Molad Y (1987) Clinical manifestations and labaratory data of subacute cutaneous lupus erythematosus. Israel J Med Sci 23: 278–280PubMedGoogle Scholar
  48. Mooney E, Wade TR (1989) Subacute cutaneous lupus erythematosus in Iceland. Int J Dermatol 28: 104–106PubMedCrossRefGoogle Scholar
  49. Nagata C, Fujita S, Iwata H, Kurosawa Y, Kobayashi K, Kobayashi M, Motegi K, Omura T, Yamamoto M, Nose T, Nakamura K-I, Hashimoto T, Nakamura Y, Yanagawa H (1995) Systemic lupus erythematosus: a case-control epidemiologic study in Japan. Int J Dermatol 34: 333–337PubMedCrossRefGoogle Scholar
  50. Nyrén O, Yin L, Josefsson S, McLaughlin JK, Blot WJ, Engqvist M, Hakelius L, Boice Jr JD, Adami H-O (1998) Risk of connective tissue disease and related disorders among women with breast implants: a nation-wide retrospective cohort study in Sweden. BMJ 316: 417422Google Scholar
  51. Park AJ, Black RJ, Sarhadi NS, Chetty U, Watson ACH (1997). Silicone gel-filled breast implants and connective tissue disease. Plast Reconstr Surg 101: 261–268Google Scholar
  52. Pelkonen P, Jalanko H, Lantto RK, Makela AC, Pietikainen MA, Savolainen HA, Verronen PM (1994) Incidence of systemic connective tissue diseases in children: A nationwide prospective study in Finland. J Rheumatol 21: 2143–6Google Scholar
  53. Perkins LL, Clark BD, Klein PJ, Cook RR (1995) A meta-analysis of breast implants and connective tissue disease. Ann Plast Surg 35: 561–570PubMedCrossRefGoogle Scholar
  54. Peterson LS, Nelson AM, Su WPD, Mason T, O’Fallon M, Gabriel SE (1997) The epidemiology of morphea (localized scleroderma) in Olmsted County 1960–1993. J Rheum 24: 7380Google Scholar
  55. Rzany B, Coresh J, Whelton PK, Petri M (1999) Risk factors for hypercreatinemia in patients with systemic lupus erythematosus ( SLE ). Lupus 8: 532–540PubMedCrossRefGoogle Scholar
  56. Rzany B, Danckwardt C, Jung M, Schuhmann M, Messer G, Bayerl C (2000) Erhöhtes Risiko von Malignomerkrankungen mit bullösem Pemphigoid. Akt Dermatol 26: 53–59Google Scholar
  57. Sanchez-Guerrero J, Liang MH, Karlson EW, Hunter DJ, Colditz GA (1995a) Postmenopausal Estrogen Therapy and the Risk for Developing Systemic Lupus Erythematosus. Ann Intern Med 122: 430–433PubMedGoogle Scholar
  58. Sanchez-Guerrero J, Colditz GA, Karlson EW, Hunter DJ, Speizer FE, Liang MH (1995b) Silicone breast implants and the risk of connective-tissue diseases and symptoms. N Engl J Med 332: 1666–1670PubMedCrossRefGoogle Scholar
  59. Schlesselmann JJ (1982) Case-Control Studies: Design, Conduct, Analysis. New York Oxford, Oxford University PressGoogle Scholar
  60. Schusterman MA, Kroll SS, Reece GP, Miller MJ, Ainslie N, Halabi S, Balch CM (1993) Incidence of autoimmune disease in patients after breast reconstruction with silicone gel implants versus autogenous tissue: a preliminary report. Ann Plast Surg 31: 1–6PubMedGoogle Scholar
  61. Shopland DR, Hartman AM, Gibson JT et al. (1996) Cigarette smoking among U.S. adults by state and region: estimates from the current population survey. J Natl Cancer Inst 88: 1748–1758PubMedCrossRefGoogle Scholar
  62. Siegel M, Lee SL (1973) The epidemiology of systemic lupus erythematosus. Arthritis Rheum 3: 1–54CrossRefGoogle Scholar
  63. Silman A, Jannini S, Symmons D, Bacon P (1988) An epidemiological study of scleroderma in the West Midlands. Br J Rheumatol 27: 286–290PubMedCrossRefGoogle Scholar
  64. Sontheimer RD, Thomas JR, Gilliam JN (1979) Subacute cutaneous lupus erythematosus - A cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol 115: 14091415Google Scholar
  65. Spiera H (1988) Scleroderma after silicone augmentation mammoplasty. JAMA 260: 236238Google Scholar
  66. Stone SP, Schroeter AL (1975) Bullous Pemphigoid and Malignant Neoplasms. Arch Dermatol 111: 991–994PubMedCrossRefGoogle Scholar
  67. Tamaki T, Mori S, Takehara K (1991) Epidemiological study of patients with systemic sclerosis in Tokyo. Arch Dermatol Res 283: 366–370PubMedCrossRefGoogle Scholar
  68. Valter I, Saretok S, Maricq HR (1997) Prevalence of Scleroderma Spectrum Disorders in the General Population of Estonia. Scand J Rheumatol 26: 419–425PubMedCrossRefGoogle Scholar
  69. Van Numen SA, Gatenby PA, Basten A (1982) Postmammoplasty connective tissue disease. Arthritis Rheum 25: 694–697CrossRefGoogle Scholar
  70. Yenning VA, Wojnarowska F (1990) The association of bullous pemphigoid and malignant disease: a case-control study. Br J Dermatol 123: 439–445CrossRefGoogle Scholar
  71. Yenning VA, Whitehead PH, Leigh IM, Allen J, Wojnarowska F (1991) The clinical expression of bullous pemphigoid is not determined by the specifity of the target antigen. Br J Dermatol 125: 561–565CrossRefGoogle Scholar
  72. Walley T, Mantgani A (1997) The UK General Practice Research Database. Lancet 350: 1097–1099PubMedCrossRefGoogle Scholar
  73. Weinstein C (1987) Lupus and non-lupus cutaneous manifestations in systemic lupus erythematosus. Aust NZ J Med 17: 501–506CrossRefGoogle Scholar
  74. Weisman MH, Vecchione TR, Albert D, Moore LT, Mueller MR (1988) CTD following breast augmentation: a preliminary report. Ann Plast Surg 82: 626–630Google Scholar
  75. Wells KE, Cruse CW, Baker JL, Daniels SM, Stern RA, Newman C, Seleznick MJ, Vasey FB, Brozena S, Albers SE, Frenske N (1994) The health status of women following cosmetic surgery. Plast Reconstr Surg 93: 907–912PubMedCrossRefGoogle Scholar
  76. Wigley FM, Miller R, Hochberg MC, Steen V (1992) Augmentation mammoplasty in patients with systemic sclerosis: data from the Baltimore Scleroderma Research Center and Pittsburgh Scleroderma Data Bank Arthritis Rheum 35: 65 http://www.cochrane.org (2000)Google Scholar
  77. Zillikens D, Weyer S, Roth A, Weidenthaler-Barth B, Hashimoto T, Bröcker EB (1995) Incidence on autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol 131: 957–958PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Wien 2001

Authors and Affiliations

  • Berthold Rzany
  • Niels Weller

There are no affiliations available

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