Linear IgA Disease
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.
KeywordsLinear IgA disease (LAD) Immunoglobuline A (IgA) Chronic bullous dermatosis of childhood (CBDC) Hemidesmosomes Basement membrane zone Dapsone
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On the Web
- DermNet NZ: http://www.dermnetnz.org/immune/linear-iga.html.