E. Anthony, R. Karl, T. Zappala
Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare autoimmune mucocutaneous disorder character- ized by subepithelial bullae, with IgA autoantibodies direc- ted 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 visi- ble on direct immunoﬂuorescence. It occurs in both adults and children, although the childhood form is most fre- quently termed “chronic bullous disease of childhood”. The clinical picture can be varied, and diagnosis is achieved via clinical, histopathological and immunopathologic exam- inations.
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 diag- noses, histopathology and management options will be dis- cussed.
An assessment of a triggering drug should always be sus- pected and ceased appropriately. Two common therapies are dapsone and sulfapyridine, which reduce the inﬂam- matory response and achieve disease remission in a vari- able period of time. In certain cases oral prednisone may need to be added to achieve control of the disease. Suc- cessful treatment of adult and childhood LABD with antibi- otics, including dicloxacillin, erythromycin, tetracycline, and trimethoprim-sulfamethoxazole has also been reported.