LABD: Linear IgA bullous dermatosis MMF: mycophenolate mofetil L inear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease with an incidence of 0.5 cases per million inhabitants per… Click to show full abstract
LABD: Linear IgA bullous dermatosis MMF: mycophenolate mofetil L inear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease with an incidence of 0.5 cases per million inhabitants per year. Pathogenic IgA autoantibodies mostly bind to a 97-kDa or 120-kDa proteolytic fragment of BP-180 extracellular domain. Most cases of LABD involve children and usually heal with dapsone within a few months. Drug-induced LABD also have a favorable course in most cases after stopping the culprit drug. However, some idiopathic LABD occurring in adult patients may be recalcitrant to dapsone and require systemic corticosteroids or immunosuppressive drugs. To the best of our knowledge, only 1 case of severe LABD and 1 case of dermatitis herpetiformis successfully treated with rituximab have been published to date. Despite the fact that dermatitis herpetiformis is a different disease from LABD, both diseases are mediated by IgA deposits. We report 2 patients with severe and recalcitrant LABD successfully treated with rituximab.
               
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