**Background of DSP Antibodies**
Desmoplakin (DSP) antibodies are autoantibodies targeting desmoplakin, a critical protein within desmosomal adhesion complexes. Desmosomes are intercellular junctions that maintain tissue integrity, particularly in skin and cardiac muscle. DSP anchors intermediate filaments (e.g., keratin) to desmosomal plaques, ensuring mechanical stability and cellular cohesion.
DSP antibodies are primarily associated with autoimmune blistering disorders. In paraneoplastic pemphigus (PNP), a severe mucocutaneous syndrome linked to malignancies, these antibodies disrupt desmosome-keratin interactions, leading to epithelial detachment and blistering. They are also implicated in rare cases of pemphigus vulgaris and pemphigus foliaceus. Beyond dermatologic conditions, DSP antibodies are detected in arrhythmogenic cardiomyopathy (ACM), where immune-mediated damage to cardiac desmosomes contributes to myocardial fibrosis and arrhythmias.
Diagnostically, DSP antibodies are identified via indirect immunofluorescence (IIF) or enzyme-linked immunosorbent assay (ELISA), often alongside other desmosomal autoantibodies (e.g., anti-desmoglein). Their presence aids in differentiating PNP from classical pemphigus and underscores the need to screen for occult tumors. In cardiology, anti-DSP positivity may signal autoimmune involvement in ACM, guiding immunomodulatory therapies.
Research continues to explore their pathogenic role, epitope targets, and clinical utility as biomarkers. Understanding DSP antibodies enhances diagnostic precision and informs targeted therapeutic strategies in both dermatology and cardiology.