Antidesmoplakin antibodies in pemphigus vulgaris

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Summary Background 

Besides being present in paraneoplastic pemphigus (PNP), circulating antidesmoplakin (DP) antibodies have been found anecdotally in other bullous diseases, including pemphigus foliaceus and pemphigus vulgaris. Objectives 

To verify how frequent anti-DP antibodies are in pemphigus vulgaris. Methods 

We studied 48 sera from patients with proven pemphigus vulgaris (29 mucosal dominant pemphigus and 19 mucocutaneous pemphigus) by indirect immunofluorescence (IIF) with rat bladder epithelium (RBE) as a substrate and by immunoblotting (IB) on human keratinocyte cultures enriched in DP. Results 

Ten sera (21%) were positive in IIF on RBE. By IB, eight sera proved to have antibodies to both DP I (250 kDa) and DP II (210 kDa), one serum had antibodies directed to DP I only, and two sera to DP II only. Conclusions 

Our data confirm that RBE is not a specific IIF substrate for the serological diagnosis of PNP. It remains a sensitive and specific substrate for the detection of anti-DP antibodies, which, in patients with pemphigus vulgaris, are probably caused by an epitope-spreading phenomenon.

Keywords: antidesmoplakin antibodies; pemphigus vulgaris; rat urinary bladder

Document Type: Research Article


Affiliations: Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata IRCCS, Rome, Italy

Publication date: April 1, 2006

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