Journal of Endocrinological Investigation

, Volume 27, Issue 5, pp 475–479

Fatal toxic epidermal necrolysis in autoimmune polyglandular syndrome Type I

  • A. Porzionato
  • S. Zancaner
  • C. Betterle
  • S. D. Ferrara
Case Report

Abstract

Autoimmune polyglandular syndrome Type I (APS I) is a disorder defined by the presence of at least two of the following diseases: Addison’s disease, hypoparathyroidism, and chronic mucocutaneous candidiasis. We present the case of a 45-yr-old woman, affected by APS I, in chronic treatment with betamethasone. She was referred to a Division of General Medicine for jaundice, ascites and peripheral edema attributed to worsening of pre-existing autoimmune chronic hepatitis. During hospitalization, the following drugs were given: Amoxicillin/Clavulanic acid and Levofloxacin for bronchopneumonia, Furosemide and Canreonate for renal impairment, Pantoprazole for gastric protection, and Itraconazole for oral candidiasis. After about a month, she developed widespread, sheet-like, epidermal detachment, with painful lesions of the conjunctiva, lips and mouth. Toxic epidermal necrolysis (TEN) was diagnosed, and the patient was transferred to a Burn Center, where she died 10 days after the first onset of cutaneous rash. Autoptic and histopathological findings (epidermal necrosis and detachment, lymphomonocytic infiltration of the dermis) confirmed the clinical diagnosis. TEN is a usually drug-induced cutaneous inflammatory disorder characterized by extensive epidermal detachment and frequent mucosal involvement. It has also been associated with immuno-mediated disorders (HIV infection, graft-vs-host disease, systemic lupus erythematosus, mixed essential cryoglobulinemia), in keeping with immuno-mediated pathogenesis. We present, to our knowledge, the first report of TEN in a patient with APS I, and suggest that some pathogenetic mechanisms of APS I are shared with TEN. We stress how such a disease can occur in an autoimmune syndrome, even during corticosteroid treatment.

Key-words

Autoimmune polyglandular syndrome toxic epidermal necrolysis corticosteroid Addison’s disease hypoparathyroidism 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Mignogna MD, Fedele S, Lo Russo L, Ruoppo E, Lo Muzio L. Polyglandular autoimmune syndrome Type II associated with oral lichen planus. Int J Dermatol 2002, 41: 244–6.PubMedCrossRefGoogle Scholar
  2. 2.
    Betterle C, Dal Pra C, Mantero F, Zanchetta R. Autoimmune adrenal insufficiency and autoimmune polyendocrine syndromes: autoantibodies, autoantigens, and their applicability in diagnosis and disease prediction. Endocr Rev 2002, 23: 327–64.PubMedCrossRefGoogle Scholar
  3. 3.
    Betterle C, Greggio NA, Volpato M. Autoimmune polyglandular syndrome Type 1. J Clin Endocrinol Metab 1998, 83: 1049–55.PubMedCrossRefGoogle Scholar
  4. 4.
    Neufeld M, MacLaren NK, Blizzard RM. Two types of autoimmune Addison’s disease associated with different polyglandular autoimmune (PGA) syndromes. Medicine 1981, 60: 355–62.PubMedCrossRefGoogle Scholar
  5. 5.
    Chen S, Sawicka J, Betterle C, et al. Autoantibodies to steroidogenic enzymes in autoimmune polyglandular syndrome, Addison’s disease, and premature ovarian failure. J Clin Endocrinol Metab 1996, 81: 1871–6.PubMedGoogle Scholar
  6. 6.
    Meyer G, Badenhoop K. Autoimmune regulator (AIRE) gene on chromosome 21: implications for autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) any more common manifestations of endocrine autoimmunity. J Endocrinol Invest 2002, 25: 804–11.PubMedCrossRefGoogle Scholar
  7. 7.
    Carboni I, Soda R, Bianchi L, Chimenti S. Chronic mucocutaneous candidiasis and alopecia areata as cutaneous expression of autoimmune polyglandular syndrome Type I. Acta Dermatol Venereol 2002, 82: 68–9.CrossRefGoogle Scholar
  8. 8.
    Oblinger E, Cibis A, Peter RU, Proebstle TM. Anogenital condylomata acuminata, mucocutaneous candidiasis, vitiligo, keratopathy and primary hypoparathyroidism in an autoimmune polyglandular syndrome Type 1. Dtsch Med Wochenschr 1997, 122: 1382–6.PubMedCrossRefGoogle Scholar
  9. 9.
    Sporkmann KH, Eickhoff R, Dominik HC. Subsepsis allergica in a patient with Type I polyglandular autoimmune syndrome. Monatsschr Kinderheilkd 1990, 138: 619–22.PubMedGoogle Scholar
  10. 10.
    Kappeler D, Troendle A, Mueller B. Localized granuloma annulare associated with autoimmune thyroid disease in a patient with a positive family history for autoimmune polyglandular syndrome Type II. Eur J Endocrinol 2001, 145: 101–2.PubMedCrossRefGoogle Scholar
  11. 11.
    Papadopoulos KI, Hornbald Y, Liljebladh H, Hallengren B. High frequency of endocrine autoimmunity in patients with sarcoidosis. Eur J Endocrinol 1996, 134: 331–6.PubMedCrossRefGoogle Scholar
  12. 12.
    Franzese A, Valerio G, Di Maio S, Iannucci MP, Bloise A, Tenore A. Growth hormone insufficiency in a girl with the autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy. J Endocrinol Invest 1999, 22: 66–9.PubMedCrossRefGoogle Scholar
  13. 13.
    Wilson PW, Buckley CE, Eisenbarth GS. Disordered immune function in patients with polyglandular failure. J Clin Endocrinol Metab 1981, 52: 284–8.PubMedCrossRefGoogle Scholar
  14. 14.
    Peterson P, Perheentupa J, Krohn KJ. Detection of candidal antigens in autoimmune polyglandular syndrome Type I. Clin Diagn Lab Immunol 1996, 3: 290–4.PubMedCentralPubMedGoogle Scholar
  15. 15.
    Betterle C, Mirakian R, Doniach D, Bottazzo GF, Riley W, MacLaren N. Antibodies to melanocytes in vitiligo. Lancet 1984, 1: 159.PubMedCrossRefGoogle Scholar
  16. 16.
    Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. New Engl J Med 1994, 331: 1272–85.PubMedCrossRefGoogle Scholar
  17. 17.
    Rappersberger K, Foedinger D. Treatment of erythema multiforme, Stevens-Johnson Syndrome, and toxic epidermal necrolysis. Dermatol Ther 2002, 15: 397–408.CrossRefGoogle Scholar
  18. 18.
    Kelly JP, Auquier A, Rzany B, et al. An international collaborative case-control study of severe cutaneous adverse reactions (SCAR). Design and methods. J Clin Epidemiol 1995, 48: 1099–108.PubMedCrossRefGoogle Scholar
  19. 19.
    Palmieri TL, Greenhalgh DG, Saffle JR, et al. A multicenter review of toxic epidermal necrolysis treated in U.S. burn centers at the end of the twentieth century. J Burn Care Rehabil 2002, 23: 87–96.PubMedCrossRefGoogle Scholar
  20. 20.
    Strom BL, Carson JL, Halpern AC, et al. A population-based study of Stevens-Johnson syndrome. Incidence and antecedent drug exposures. Arch Dermatol 1991, 127: 831–8.PubMedCrossRefGoogle Scholar
  21. 21.
    McCormack JG. Mycoplasma pneumoniae and the erythema multiforme Stevens-Johnson syndrome. J Infect 1981, 3: 32–6.PubMedCrossRefGoogle Scholar
  22. 22.
    Roujeau JC, Chosidow O, Saiag P, Guillaume JC. Toxic epidermal necrolysis (Lyell syndrome). J Am Acad Dermatol 1990, 23: 1039–58.PubMedCrossRefGoogle Scholar
  23. 23.
    Roujeau JC, Huynh TN, Bracq C, Guillaume JC, Revuz J, Touraine R. Genetic susceptibility to toxic epidermal necrolysis. Arch Dermatol 1987, 123: 1171–3.PubMedCrossRefGoogle Scholar
  24. 24.
    Burge SM, Dawber RPR. Stevens-Johnson syndrome and toxic epidermal necrolysis in a patient with systemic lupus erythematosus. J Am Acad Dermatol 1985, 13: 665–6.PubMedCrossRefGoogle Scholar
  25. 25.
    Coopman SA, Johnson RA, Platt R, Stern RS. Cutaneous disease and drug reactions in HIV infection. New Engl J Med 1993, 328: 1670–4.PubMedCrossRefGoogle Scholar
  26. 26.
    Solinas A, Cottoni F, Tanda F, Tocco A. Toxic epidermal necrolysis in a patient affected by mixed essential cryoglobulinemia. J Am Acad Dermatol 1988, 18: 1165–9.PubMedCrossRefGoogle Scholar
  27. 27.
    Rzany B, Schmitt H, Schopf E. Toxic epidermal necrolysis in patients receiving glucocorticosteroids. Acta Dermatol Venereol 1991, 71: 171–2.Google Scholar
  28. 28.
    Roujeau JC, Kelly JP, Naldi L, et al. Medication use and risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. New Engl J Med 1995, 333: 1600–07.PubMedCrossRefGoogle Scholar

Copyright information

© Italian Society of Endocrinology (SIE) 2004

Authors and Affiliations

  • A. Porzionato
    • 1
  • S. Zancaner
    • 1
  • C. Betterle
    • 2
  • S. D. Ferrara
    • 1
  1. 1.Department of Environmental Medicine and Public Health, Forensic Toxicology and Antidoping Unit, Section of Legal MedicineUniversity of PaduaPaduaItaly
  2. 2.Endocrine Unit, Department of Medical and Surgical SciencesUniversity of PaduaPaduaItaly

Personalised recommendations