Autoantibodies Against Desmoplakin I and II in Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis / 대한피부과학회지

ABSTRACT

BACKGOUND It has been suggested that humoral immune mechanisms might play a role in the pathogenesis of a subset of patients with Stevens-Johnson syndrome (SJS). Circulating antibodies (Abs) against desmoplakin I and II (dp I/II) were detected in a subset of patients with SJS, which could impair the function of desmosome-keratinfilament complexes resulting in suprabasal acantholysis as a humoral autoimmune phenomenon. OBJECTIVE: The purpose of this study was to detect the presence of circulating autoantibodies against dp I/II in the sera of patients with toxic epidermal necrolysis (TEN), as well as SJS. METHOD: In this study, the sera of ten patients with TEN and SJS were investigated. Immunoblot analyses of extracts of EDTA-separated normal human epidermis were performed. We also performed an indirect immunofluorescence (IIF) test using normal human skin, mouse tongue, esophagus, and rat bladder. In addition, we performed direct IF studies using three perilesional tissues obtained from one patient of SJS and two patients of TEN. RESULTS: By immunoblotting using human epidermal extract, 215-kD and 250-kD proteins were detected in the sera of six out of ten patients with TEN and SJS. IIF using normal human foreskin demonstrated dense IgG deposits in the intercellular spaces and cytoplasmic membrane of epidermal cells in all sera of patients with SJS and TEN. In the direct IF test, IgG and IgA deposits appeared in an intercellular staining of epidermis of perilesional skin from two out of three patients with TEN and SJS. CONCLUSION: These findings suggest that circulating Abs against dp I/II and constitutive desmosomal plaque proteins might play a role in the pathogenesis of SJS and TEN.