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Blistering Diseases pp 299–305Cite as

IgA Pemphigus

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Abstract

IgA pemphigus is a less life-threatening subtype of pemphigus. IgA pemphigus is characterized clinically by flaccid pustules and erosions and histopathologically by minimal acantholysis and extensive neutrophilic infiltration in either the subcorneal or entire epidermis. Immunologically, IgA pemphigus is characterized by the presence of IgA autoantibodies. IgA pemphigus is subdivided into two types: subcorneal pustular dermatosis-type IgA pemphigus (SPD-type IgA pemphigus) and intraepidermal neutrophilic IgA dermatosis-type IgA pemphigus (IEN-type IgA pemphigus). The autoantigen of the former is desmocollin-1, a transmembrane constituent of desmosomes, and that of the latter is currently unknown.

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Abbreviations

IEN:

Intraepidermal neutrophilic

SPD:

Subcorneal pustular dermatosis

References

  1. Tsuruta D, Ishii N, Hamada T, Ohyama B, Fukuda S, Koga H, et al. IgA pemphigus. Clin Dermatol. 2011;29(4):437–42.

    Article  PubMed  Google Scholar 

  2. Hashimoto T, Ebihara T, Nishikawa T. Studies of autoantigens recognized by IgA anti-keratinocyte cell surface antibodies. J Dermatol Sci. 1996;12(1):10–7.

    Article  CAS  PubMed  Google Scholar 

  3. Hashimoto T, Yasumoto S, Nagata Y, Okamoto T, Fujita S. Clinical, histopathological and immunological distinction in two cases of IgA pemphigus. Clin Exp Dermatol. 2002;27(8):636–40.

    Article  CAS  PubMed  Google Scholar 

  4. Hashimoto T, Kiyokawa C, Mori O, Miyasato M, Chidgey MA, Garrod DR, et al. Human desmocollin 1 (Dsc1) is an autoantigen for the subcorneal pustular dermatosis type of IgA pemphigus. J Invest Dermatol. 1997;109(2):127–31.

    Article  CAS  PubMed  Google Scholar 

  5. Wallach D. Intraepidermal IgA pustulosis. J Am Acad Dermatol. 1992;27(6 Pt 1):993–1000.

    Article  CAS  PubMed  Google Scholar 

  6. Gengoux P, Tennstedt D, Lachapelle JM. Intraepidermal neutrophilic IgA dermatosis: pemphigus-like IgA deposits. Dermatology. 1992;185(4):311–3.

    Article  CAS  PubMed  Google Scholar 

  7. Teraki Y, Amagai N, Hashimoto T, Kusunoki T, Nishikawa T. Intercellular IgA dermatosis of childhood. Selective deposition of monomer IgA1 in the intercellular space of the epidermis. Arch Dermatol. 1991;127(2):221–4.

    Article  CAS  PubMed  Google Scholar 

  8. Hashimoto T, Ebihara T, Dmochowski M, Kawamura K, Suzuki T, Tsurufuji S, et al. IgA antikeratinocyte surface autoantibodies from two types of intercellular IgA vesiculopustular dermatosis recognize distinct isoforms of desmocollin. Arch Dermatol Res. 1996;288(8):447–52.

    CAS  PubMed  Google Scholar 

  9. Chan LS. Pemphigus, IgA. emedicine. Available at: http://www.emedicine.medscape.com/article/1063776-overview. Accessed April 9, 2010.

  10. Chorzelski TP, Beutner EH, Kowalewski C, Olszewska M, Maciejowska E, Seferowicz E, et al. IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. J Am Acad Dermatol. 1991;24(5 Pt 2):839–44.

    Article  CAS  PubMed  Google Scholar 

  11. Erdag G, Qureshi HS, Greer KE, Patterson JW. Immunoglobulin A pemphigus involving the perianal skin and oral mucosa: an unusual presentation. Cutis. 2007;80(3):218–20.

    PubMed  Google Scholar 

  12. Hodak E, David M, Ingber A, Rotem A, Hazaz B, Shamai-Lubovitz O, et al. The clinical and histopathological spectrum of IgA-pemphigus—report of two cases. Clin Exp Dermatol. 1990;15(6):433–7.

    Article  CAS  PubMed  Google Scholar 

  13. Ishii N, Ishida-Yamamoto A, Hashimoto T. Immunolocalization of target autoantigens in IgA pemphigus. Clin Exp Dermatol. 2004;29(1):62–6.

    Article  CAS  PubMed  Google Scholar 

  14. Kim SC, Won JH, Chung J, Bang DS. IgA pemphigus: report of a case with immunoelectron localization of bound IgA in the skin. J Am Acad Dermatol. 1996;34(5 Pt 1):852–4.

    Article  CAS  PubMed  Google Scholar 

  15. Yasuda H, Kobayashi H, Hashimoto T, Itoh K, Yamane M, Nakamura J. Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review. Br J Dermatol. 2000;143(1):144–8.

    Article  CAS  PubMed  Google Scholar 

  16. Karpati S, Amagai M, Liu WL, Dmochowski M, Hashimoto T, Horvath A. Identification of desmoglein 1 as autoantigen in a patient with intraepidermal neutrophilic IgA dermatosis type of IgA pemphigus. Exp Dermatol. 2000;9(3):224–8.

    Article  CAS  PubMed  Google Scholar 

  17. Hashimoto T, Komai A, Futei Y, Nishikawa T, Amagai M. Detection of IgA autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus. Arch Dermatol. 2001;137(6):735–8.

    CAS  PubMed  Google Scholar 

  18. Endo Y, Tsujioka K, Tanioka M, Minegaki Y, Ohyama B, Hashimoto T, et al. Bullous dermatosis associated with IgG antibodies specific for desmocollins. Eur J Dermatol. 2010;20(5):620–5.

    PubMed  Google Scholar 

  19. Zaraa I, Kerkeni N, Sellami M, Chelly I, Zitouna M, Makni S, et al. IgG/IgA pemphigus with IgG and IgA antidesmoglein 3 antibodies and IgA antidesmoglein 1 antibodies detected by enzyme-linked immunosorbent assay: a case report and review of the literature. Int J Dermatol. 2010;49(3):298–302.

    Article  PubMed  Google Scholar 

  20. Kopp T, Sitaru C, Pieczkowski F, Schneeberger A, Fodinger D, Zillikens D, et al. IgA pemphigus—occurrence of anti-desmocollin 1 and anti-desmoglein 1 antibody reactivity in an individual patient. J Dtsch Dermatol Ges. 2006;4(12):1045–50.

    Article  PubMed  Google Scholar 

  21. Duker I, Schaller J, Rose C, Zillikens D, Hashimoto T, Kunze J. Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. Arch Dermatol. 2009;145(10):1159–62.

    Article  PubMed  Google Scholar 

  22. Carayannopoulos L, Hexham JM, Capra JD. Localization of the binding site for the monocyte immunoglobulin (Ig) A-Fc receptor (CD89) to the domain boundary between Calpha2 and Calpha3 in human IgA1. J Exp Med. 1996;183(4):1579–86.

    Article  CAS  PubMed  Google Scholar 

  23. Petropoulou H, Politis G, Panagakis P, Hatziolou E, Aroni K, Kontochristopoulos G. Immunoglobulin a pemphigus associated with immunoglobulin A gammopathy and lung cancer. J Dermatol. 2008;35(6):341–5.

    Google Scholar 

  24. Camisa C, Warner M. Treatment of pemphigus. Dermatol Nurs. 1998;10(2):115–8, 23–31.

    CAS  PubMed  Google Scholar 

  25. McLeish KR, Miller FN, Stelzer GT, Wellhausen SR. Mechanism by which methylprednisolone inhibits acute immune complex-induced changes in vascular permeability. Inflammation. 1986;10(3):321–32.

    Article  CAS  PubMed  Google Scholar 

  26. Williams RN, Paterson CA. The influence of topical corticosteroid therapy upon polymorphonuclear leukocyte distribution, vascular integrity and ascorbate levels in endotoxin-induced inflammation of the rabbit eye. Exp Eye Res. 1987;44(2):191–8.

    Article  CAS  PubMed  Google Scholar 

  27. Zurier RB, Weissman G. Anti-immunologic and anti-inflammatory effects of steroid therapy. Med Clin N Am. 1973;57(5):1295–307.

    CAS  PubMed  Google Scholar 

  28. Gruss C, Zillikens D, Hashimoto T, Amagai M, Kroiss M, Vogt T, et al. Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin. J Am Acad Dermatol. 2000;43(5 Pt 2):923–6.

    Article  CAS  PubMed  Google Scholar 

  29. Ruiz-Genao DP, Hernandez-Nunez A, Hashimoto T, Amagai M, Fernandez-Herrera J, Garcia-Diez A. A case of IgA pemphigus successfully treated with acitretin. Br J Dermatol. 2002;147(5):1040–2.

    Article  CAS  PubMed  Google Scholar 

  30. Howell SM, Bessinger GT, Altman CE, Belnap CM. Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. J Am Acad Dermatol. 2005;53(3):541–3.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Dermatology, Osaka City University Graduate School of Medicine1-4-3 Asahimachi, Abeno-ku, Osaka, 545-8585, Japan

    Daisuke Tsuruta MD, PhD

  2. Department of Dermatology, Kurume University School of Medicine and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan

    Takashi Hashimoto MD

Authors
  1. Daisuke Tsuruta MD, PhD
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  2. Takashi Hashimoto MD
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Correspondence to Daisuke Tsuruta MD, PhD .

Editor information

Editors and Affiliations

  1. Department of Dermatology, St George Hospital, University of New South Wales, Sydney, New South Wales, Australia

    Dédée F. Murrell

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Tsuruta, D., Hashimoto, T. (2015). IgA Pemphigus. In: Murrell, D. (eds) Blistering Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45698-9_26

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  • DOI: https://doi.org/10.1007/978-3-662-45698-9_26

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