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Linear immunoglobulin A bullous dermatosis

  • Giulio Fortuna
    Affiliations
    Department of Dermatology, Program in Epithelial Biology, Stanford University School of Medicine, 269 Campus Dr, No. 2145, Stanford, CA 94305

    Oral Medicine Unit, Department of Odontostomatological and Maxillofacial Sciences, Federico II University of Naples, Via Pansini 5, 80131 Naples, Italy
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  • M. Peter Marinkovich
    Correspondence
    Corresponding author. Tel.: +1 650 498 5425; fax: +1 650 723 8762.
    Affiliations
    Department of Dermatology, Program in Epithelial Biology, Stanford University School of Medicine, 269 Campus Dr, No. 2145, Stanford, CA 94305

    Veterans Affairs Palo Alto Healthcare System, 3801 Miranda Ave, Palo Alto, CA 94304
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      Abstract

      Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is an autoimmune mucocutaneous disorder characterized by subepithelial bullae, with IgA autoantibodies directed against several different antigens in the basement membrane zone. Its immunopathologic characteristic resides in the presence of a continuous linear IgA deposit along the basement membrane zone, which is clearly visible on direct immunofluorescence. This disorder shows different clinical features and distribution when adult-onset of linear IgA disease is compared with childhood-onset. Diagnosis is achieved via clinical, histopathologic, and immunopathologic examinations. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time.
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