TY - JOUR
T1 - Anti-desmoplakin antibodies in erythema multiforme and Stevens-Johnson syndrome sera
T2 - Pathogenic or epiphenomenon?
AU - Cozzani, Emanuele
AU - di Zenzo, Giovanni
AU - Calabresi, Valentina
AU - Caproni, Marzia
AU - Schena, Donatella
AU - Quaglino, Pietro
AU - Marzano, Angelo V.
AU - Fabbri, Paolo
AU - Rebora, Alfredo
AU - Parodi, Aurora
PY - 2011/1
Y1 - 2011/1
N2 - The pathophysiology of erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) is unclear. Whether autoantibodies against desmoplakin (Dp) I and II play a pathogenic role or result from an epitope spreading phenomenon is uncertain. Our aim was to characterize the keratinocyte antigens recognized in EM, TEN and SJS. Of 33 patients studied, 2 had TEN, 1 SJS, 9 EM major and 21 EM minor, according to Roujeau's criteria. All sera were studied by indirect immunofluorescence (IIF), immunoblotting and immunoprecipitation. Twenty normal sera were used as controls. 10/33 sera reacted with polypeptides of 215 and/or 250-kDa molecular mass, which co-migrate with Dp I and II as assessed by an anti-Dp I and II monoclonal antibody on IB. In IP, none of the anti-Dp I and -Dp II 10 patient sera immunoprecipitated Dp I and/or II from radiolabeled keratinocyte extracts. Two of 10 patient sera (SJS, EM minor) reacted with DpI and II when denaturated by the IB procedure. The reactivity against intracellular antigens DpI and II as denaturated proteins may result from the epidermal damage produced by aggressive autoreactive T cells, playing therefore only a secondary role in the pathogenesis of the disease.
AB - The pathophysiology of erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) is unclear. Whether autoantibodies against desmoplakin (Dp) I and II play a pathogenic role or result from an epitope spreading phenomenon is uncertain. Our aim was to characterize the keratinocyte antigens recognized in EM, TEN and SJS. Of 33 patients studied, 2 had TEN, 1 SJS, 9 EM major and 21 EM minor, according to Roujeau's criteria. All sera were studied by indirect immunofluorescence (IIF), immunoblotting and immunoprecipitation. Twenty normal sera were used as controls. 10/33 sera reacted with polypeptides of 215 and/or 250-kDa molecular mass, which co-migrate with Dp I and II as assessed by an anti-Dp I and II monoclonal antibody on IB. In IP, none of the anti-Dp I and -Dp II 10 patient sera immunoprecipitated Dp I and/or II from radiolabeled keratinocyte extracts. Two of 10 patient sera (SJS, EM minor) reacted with DpI and II when denaturated by the IB procedure. The reactivity against intracellular antigens DpI and II as denaturated proteins may result from the epidermal damage produced by aggressive autoreactive T cells, playing therefore only a secondary role in the pathogenesis of the disease.
KW - Autoantibodies
KW - Desmoplakins
KW - Erythema multiforme
KW - Immunoblotting
KW - Immunofluorescence
KW - Pathogenesis
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U2 - 10.1684/ejd.2010.1150
DO - 10.1684/ejd.2010.1150
M3 - Article
C2 - 21233064
AN - SCOPUS:79957502073
VL - 21
SP - 32
EP - 36
JO - European Journal of Dermatology
JF - European Journal of Dermatology
SN - 1167-1122
IS - 1
ER -