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Linear IgA Disease

  • Barbara HorváthEmail author
  • Marcel F. Jonkman
Chapter

Abstract

Linear IgA disease (LAD) is a group of heterogeneous autoimmune subepidermal bullous diseases characterized by exclusively IgA autoantibodies targeting a component of the epidermal basement membrane zone. The lamina lucida-type LAD, also known as chronic bullous dermatosis of childhood, is a rare disease yet the most common autoimmune bullous disease among children with a peak incidence at 4–5 years of age. The major target antigens in lamina lucida-type LAD are 120 kDa LAD-1 antigen and its carboxyterminal proteolysed form 97 kDa LAD antigen 1 (LABD97). Both proteins are produced by cleavage of the extracellular domain of BP180, one of the main structural components of the hemidesmosome. The autoantigen targeted by IgA in the sublamina densa-type LAD, also known as IgA epidermolysis bullosa acquisita, is type VII collagen. In adults, LAD may also be drug induced.

Keywords

Linear IgA disease (LAD) Immunoglobuline A (IgA) Chronic bullous dermatosis of childhood (CBDC) Hemidesmosomes Basement membrane zone Dapsone 

References

  1. 1.
    Kasperkiewicz M, Zillikens D, Schmidt E. Pemphigoid diseases: pathogenesis, diagnosis, and treatment. Autoimmunity. 2012;45:55–70.CrossRefPubMedGoogle Scholar
  2. 2.
    Lings K, Bygum A. Linear IgA bullous dermatosis: a retrospective study of 23 patients in Denmark. Acta Derm Venereol. 2015;95:466-71.Google Scholar
  3. 3.
    Jablonska S, Chorzelski TP, Rosinska D, Maciejowska E. Linear IgA bullous dermatosis of childhood (chronic bullous dermatosis of childhood). Clin Dermatol. 1991;9:393–401.CrossRefPubMedGoogle Scholar
  4. 4.
    Zone JJ, Egan CA, Taylor TB, Meyer LJ. IgA autoimmune disorders: development of a passive transfer mouse model. J Investig Dermatol Symp Proc. 2004;9:47–51.CrossRefPubMedGoogle Scholar
  5. 5.
    Egan CA, Zone JJ. Linear IgA bullous dermatosis. Int J Dermatol. 1999;38:818–27.CrossRefPubMedGoogle Scholar
  6. 6.
    Chanal J, Ingen-Housz-Oro S, Ortonne N, Duong TA, Thomas M, Valeyrie-Allanore L, Lebrun-Vignes B, André C, Roujeau JC, Chosidow O, Wolkenstein P. Linear IgA bullous dermatosis: comparison between the drug-induced and spontaneous forms. Br J Dermatol. 2013;169:1041–8.CrossRefPubMedGoogle Scholar
  7. 7.
    Wojnarowska F, Bhogal BS, Black MM. Chronic bullous disease of childhood and linear IgA disease of adults are IgA1-mediated diseases. Br J Dermatol. 1994;131:201–4.CrossRefPubMedGoogle Scholar
  8. 8.
    Kuechle MK, Stegemeir E, Maynard B, Gibson LE, Leiferman KM, Peters MS. Drug-induced linear IgA bullous dermatosis: report of six cases and review of the literature. J Am Acad Dermatol. 1994;30:187–92.CrossRefPubMedGoogle Scholar
  9. 9.
    Kasperkiewicz M, Schmidt E. Current treatment of autoimmune blistering diseases. Curr Drug Discov Technol. 2009;6:270–80.CrossRefPubMedGoogle Scholar
  10. 10.
    Guide SV, Marinkovich MP. Linear IgA bullous dermatosis. Clin Dermatol. 2001;19:719–27.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of DermatologyCenter for Blistering Diseases, University Medical Center Groningen, University of GroningenGroningenthe Netherlands

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