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KMID : 0363219920300040467
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Korean Journal of Dermatology
1992 Volume.30 No. 4 p.467 ~ p.477
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Immunopathologica Studies in Pemphigus Vulaaris, Bullous Pemphigoid and Epidermolysis Bullosa Acqusita-Subclass distribution of IgG autoantibodies and in vitro complement immunofluorescence
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Abstract
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Pemphgus vulgaris (PV), Bullus pemphigoid (BP), and Epidermolysis bullous acqusita (EBA) are autoimmune bullous dermatoses, characterized by circulating IgG autoantibodies. These antibodies react with antigens located at the intercellular
substance
(ICS) of epidermis, basement membrane zone (BMZ), and subepidermal anchoring fibril zone (AFZ), respectively. The subclass distribution of IgG autoantibodies, and the properties and degrees of complement fixing activities of these autoantibodies
in
each
of the above diseases have not been well understood.
Indirect immunofluorescence and in vitro complement stainings were performed for the titration of subclasses of IgG antibodies and for the immunofluorescence staining reactivities of complement components C3, C4, C5b-9, H, C4bp, and S. Each serum
specimen from five cases of PV, five cases of BP. and three cases of EBA was tested. The findings of multistep technique with monoclonal and polyclonal antibodies are as follows :
All four subclasses of IgG antibodies were identified at the antigenic sites in these group, however there were some differences in the antibodies titers. In PV and BP the dominant subclass of highest antibody titer was IgG1 and/or IgG4. In EBA
only
IgG4 was dominant in all three cases. The results of complement component stainings, in most of the cases of PV, showed positive for C3 and C4 but were negative for the other components or inhibitor proteins at the ICS of epidermis. In BP most of
the
cases revealed positive staining reactivities at the BMZ for C3, C4, C5b-9, H, and C4bp-9 with no staining reactivities for the inhibitor proteins
No significant relevancy was found between the titers of complement fixing IgG subclasses and the numbers of positive complement staining reactivities for complement components. The results suggest that the complement system may contribute more
strongly
to the formation of bullous lesions in BP and EBA than in PV. (Kor J Dermatol 1992;30 (4) : 467-477)
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