II. Epidemiology

  1. Onset over age 30 years

III. Pathophysiology

  1. Autoimmune bullous disorder
  2. Variant of Chronic Bullous Disease of Childhood

IV. Symptoms

V. Signs

  1. Annular Lesions
    1. Papules (may be excoriated and crusted)
    2. Vesicles
    3. Bullae
  2. Distribution
    1. Symmetric involvement on extensor surfaces
    2. Elbows, knees, and buttocks affected
    3. Mucusa involvement may occur (Conjunctiva, oral)

VI. Labs

  1. Histology
    1. Subepidermal Blisters
    2. Inflammatory infiltrates at basement membrane zone
  2. Immunofluorescence
    1. IgA deposition along basement membrane

VII. Management

  1. First Line: Dapsone or Sulfapyridine
    1. See Dapsone (requires G6PD and monitoring of CBC)
  2. Adjunct: Low dose Prednisone initially

VIII. Course

  1. Variable course that may last for years

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Ontology: Linear IgA Bullous Dermatosis (C0406650)

Definition (MSH) Autoimmune disease characterized by subepidermal blisters and linear deposition of autoantibodies at the dermoepidermal junction. The accumulated autoantibodies are of IMMUNOGLOBULIN A and occasionally IMMUNOGLOBULIN G classes against epidermal BASEMENT MEMBRANE proteins. The dermatosis is sometimes associated with malignancies and use of certain drugs (e.g., VANCOMYCIN).
Concepts Disease or Syndrome (T047)
MSH D062027
SnomedCT 95330001
English Linear IgA bullous dermatosis, linear IgA dermatoses (diagnosis), linear IgA dermatoses, linear IgA dermatosis, Linear Ig A disease, Linear IgA, linear iga bullous dermatosis, dermatosis iga linear, linear iga, linear iga disease, linear iga dermatosis, IgA Dermatoses, Linear, Linear IgA Dermatoses, Linear IgA Bullous Dermatosis, Linear IgA Dermatosis, Dermatosis, Linear IgA, IgA Dermatosis, Linear, Dermatoses, Linear IgA, Linear IgA Bullous Dermatosis [Disease/Finding], Linear immunoglobulin A dermatosis (disorder), Linear IgA dermatosis (disorder), Linear immunoglobulin A dermatosis, IgA - Linear immunoglobulin A bullous dermatosis, LAD - Linear IgA disease, Linear IgA dermatosis, Linear IgA disease, Linear immunoglobulin A bullous dermatosis
Dutch lineaire IgA bulleuze dermatose, lineaire IgA, lineaire IgA ziekte
French IgA linéaire, Dermatose à IgA linéaire, Dermatose bulleuse linéaire à IgA, Dermatose linéaire à IgA, Dermatose bulleuse à dépôts linéaires d'IgA, Dermatose à dépôts linéaires d'IgA, Dermatose bulleuse à IgA linéaire, Maladie par dépôts linéaires d'IgA
German lineare blasenbildende IgA-Dermatose, lineare IgA, Lineare IgA-Dermatose, lineare IgA-Erkrankung
Portuguese IgA linear, Dermatose bolhosa IgA linear, Dermatose Linear Bolhosa por IgA, Dermatose Bolhosa Linear por IgA, Doença IgA linear
Spanish IgA lineal, Dermatosis bullosa linear mediada por IgA, Dermatosis Bullosa IgA Lineal, dermatosis lineal por inmunoglobulina A, dermatosis lineal por IgA, dermatosis lineal por inmunoglobulina A (trastorno), Enfermedad de la IgA lineal
Japanese リニアIgA, リニアIgA水疱性皮膚症, リニアIgA病, リニアIgAスイホウセイヒフショウ, リニアIgAビョウ, リニアIgA
Czech Lineární IgA, Lineární IgA choroba, Lineární IgA bulózní dermatóza, LABD, lineární IgA dermatóza, lineární IgA bulózní dermatóza
Italian Malattia da IgA lineari, IgA lineari, Dermatosi bollosa da IgA lineari, Dermatosi bollosa a IgA lineari
Hungarian Linearis Ig A betegség, Lineáris IgA, Lineáris Ig A betegség, Lineáris Ig A bullosus dermatosis
Polish Dermatoza pęcherzowa linijna IgA, Linijna dermatoza pęcherzowa, Dermatoza pęcherzowa z linijnym odkładaniem IgA
Russian ЛЕКАРСТВОМ ИНДУЦИРОВАННЫЙ ЛИНЕЙНЫЙ IGA-БУЛЛЕЗНЫЙ ДЕРМАТОЗ, ДЕРМАТОЗ ЛИНЕЙНЫЙ IGA/IGG, DERMATOZ LINEINYI IGA/IGG, LEKARSTVOM INDUTSIROVANNYI LINEINYI IGA-BULLEZNYI DERMATOZ, KHRONICHESKOE BULLEZNOE ZABOLEVANIE U DETEI, ЛИНЕЙНЫЙ IGA-ЗАВИСИМЫЙ БУЛЛЕЗНЫЙ ДЕРМАТОЗ, ХРОНИЧЕСКОЕ БУЛЛЕЗНОЕ ЗАБОЛЕВАНИЕ У ДЕТЕЙ, LINEINYI IGA-ZAVISIMYI BULLEZNYI DERMATOZ, ЛИНЕЙНЫЙ IGA БУЛЛЕЗНЫЙ ДЕРМАТОЗ, LINEINYI IGA BULLEZNYI DERMATOZ
Norwegian Lineær IgA bulløs dermatose