Linear IgA bullous dermatosis

Linear IgA bullous dermatosis

Linear IgA bullous dermatosis
This is a digital copy of a book that was preserved for generations on library shelves before it was carefully scanned by Google as part of a project to make the world’s books discoverable online. Whether autoimmunity is also reflected by the presence of circulating autoantigen-specific memory B cells is still a matter of debate. In this issue of JID, Cristina Has and an international group of specialists on epidermolysis bullosa (EB) report on a substitution mutation of collagen XVII that is associated with several unexpected skin and mucosal features that are ordinarily not observed in patients with junctional EB other (non-Herlitz-type junctional EB) disease. Twenty-two of 22 (100%) BP sera that immunoblotted BP180 in keratinocyte extracts, bound a mutant form consisting of the entire ECD of BP180, whereas only three of these 22 sera (14%) reacted against the ECD of BP180 lacking the NC16A membrane proximal region. This immunolabeling pattern was associated with hemidesmosomes and localized at a mean distance of 28 nm beneath the plasma membrane of basal keratinocytes. The 20 microsequenced N-terminal amino acids exhibited no homology to known basement membrane proteins but exhibited a 70% homology to a 90-kDa tumor-associated antigen. When patients with ocular disease were excluded, however, the increased occurrence of the DQB1*0301 allele in patients with oral disease was not statistically significant (64%, 7/11, p < 0.25). All 15 bullous pemphigoid sera tested reacted with a recombinant protein containing this BP180 segment. These results suggest factors in addition to C-binding titers are important in the activation of C by BP and EBA immune complexes and suggest chemotactic factors other than those derived from C activation may be important in the recruitment of leukocytes in BP. Adults frequently develop LABD in the later stages of life, with many cases occurring after the age of 60 [2,3]. This molecular analysis predicts that the BP180 antigen is an integral membrane protein of the hemi-desmosome that contains a long extracellular collagenous tail. Rarely, neonates are affected [5,6].

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