A Comparative Study for the Titer of IgG Autoantibodies in the Serum and Urine of Patients with Bullous Pemphigoid, Epidermolysis Bullosa Acqusita, and Pemphigus / 대한피부과학회지

BACKGROUND: The immunoglobulins can be detected in the urine in normal people without any renal disease. According to recent articles, it is known that autoantibodies of IgG, which share the identical antigenic specificities with the serum autoantibodies, targeting some nuclear antigens and cutaneous basement membrane zone antigens could be detected in the urine in patients with systemic lupus erythematosus and autoimmune bullous diseases. OBJECTIVE: We examined the urine for the detection of autoantibodies in patients with autoimmune bullous diseases and compared the titers of IgG autoantibodies in the serum and urine in each patient. Patients and METHODS: Nine patients were included in this study. They were 3 patient-groups of bullous pemphigoid(BP), epidermolysis bullosa acqusita(EBA), and pemphigus foliaceus(PF). Each group consisted of 3 patients having circulating autoantibodies in the serum. For a semi-quantitation of the IgG autoantibodies in the serum and urine specimens in each group, the indirect immuno fluorescence(IIF) examinations were performed. RESULTS: Seven out of nine patients showed positive results of autoantibodies in urine specimen. In the BP and EBA group, IgG autoantibodies were detected in the urine of all six patients at low titers. In the PF group, one out of three patients showed IgG autoantibodies in the urine. The high antibody titers in the sera roughly correlated with the positivity/titer of the urine IgG autoantibodies. CONCLUSION: The IIF examination of the urine could be tried for a diagnosis of autoimmune bullous diseases in some patients with active autoimmune bullous dermatoses, espacially in cases with certain difficulties in collecting serum samples.

Depositions of Complement Components and Their Inhibitors in Atuto - immune Dermatoses / 대한피부과학회지

The complement system is known to be involved in the pathogenesis of the skin lesions in pernphigus vulgaris, bullous pemphigoid, dermatitis herpetiformis, epidermolysis bullosa acquisita, and systemic lupus erythematosus. Authors examined the skin specimens of each disease cases, who did not show any evidence of complement deficiency, to determine the deposition of complement components(C4, C3, Chb-9) and their inhibitors(C4bp, Factor H, S-protein) by modified direct immunofluorescence. We also looked at the staining pattern and localization, for further insights of their pathobiologic contributions in each disease. The findings of deposits of complement components up to C9, as well as inhibitor proteins at the primary histopathologic sites, in the majority of those cases, may indicate that the complement system, to certain extent, involves the inflamrnatory reactions in these diseases. The co-localization of C5b-9 and S-protein could be regarded as the consequence of in situ formation of SC5b-9 complexs or as the result of non-lytic adsorbed complexes of fluid phase SC5b-9. The pathologic role of the complement seems to depend mostly on the complement-fixing biologic property and the amount of the tissue bound immune complexes, which are often heterogeneous to different diseases and among different patients.