Linear IgA bullous dermatosis

Abstract

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare 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. It occurs in both adults and children, although the childhood form is most frequently termed “chronic bullous disease of childhood”. The clinical picture can be varied, and diagnosis is achieved via clinical, histopathological and immunopathologic examinations. Two illustrative cases are described. A 71 year old female with vancomycin induced LABD, and a 4 year old girl with chronic bullous disease of childhood. The differential diagnoses, histopathology and management options will be discussed. An assessment of a triggering drug should always be suspected and ceased appropriately. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time. In certain cases oral prednisone may need to be added to achieve control of the disease. Successful treatment of adult and childhood LABD with antibiotics, including dicloxacillin, erythromycin, tetracycline, and trimethoprim-sulfamethoxazole has also been reported.L723041262016-06-29 <br />