TY - CHAP M1 - Book, Section TI - Intercellular Immunoglobulin (Ig) A Dermatosis (IgA Pemphigus) A1 - Hashimoto, Takashi A2 - Kang, Sewon A2 - Amagai, Masayuki A2 - Bruckner, Anna L. A2 - Enk, Alexander H. A2 - Margolis, David J. A2 - McMichael, Amy J. A2 - Orringer, Jeffrey S. PY - 2019 T2 - Fitzpatrick's Dermatology, 9e AB - AT-A-GLANCEIntercellular immunoglobulin (Ig) A dermatosis (IAD) is a chronic vesicular and/or pustular autoimmune skin disease caused by IgA, not by IgG, antibodies.Two major types: subcorneal pustular dermatosis (SPD)-type IAD and intraepidermal neutrophilic IgA dermatosis (IEN)-type IAD.SPD-type IAD clinically shows superficial pustules on the intertriginous areas.IEN-type IAD clinically shows atypical pustular skin lesions with a sunflower-like configuration.Diagnosis is made by histopathology showing intraepidermal pustules, immunofluorescence detecting in vivo bound and/or circulating IgA antikeratinocyte cell-surface autoantibodies, and various biochemical and molecular biologic methods demonstrating reactivity with various autoantigens.SPD-type IAD shows histopathologically subcorneal neutrophilic pustules.IEN-type IAD shows histopathologically neutrophilic pustules in the middle epidermis.Major autoantigens are desmogleins (Dsg) and desmocollins (Dsc), cadherin-type cell-to-cell adhesion molecules found in desmosomes.Patients are treated mainly with dapsone and systemic corticosteroids; other treatment options include immunosuppressive agents, tetracycline, colchicine, plasmapheresis, retinoids, adalimumab, and psoralen and ultraviolet A. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - dermatology.mhmedical.com/content.aspx?aid=1161331806 ER -