Clinical activity of pemphigus vulgaris relates to IgE autoantibodies against desmoglein 3

Nagel, Angela; Lang, Angelika; Engel, Dorothee; Podstawa, Eva; Hunzelmann, Nicolas; de Pita, Ornella; Borradori, Luca; Uter, Wolfgang; Hertl, Michael (2010). Clinical activity of pemphigus vulgaris relates to IgE autoantibodies against desmoglein 3. Clinical immunology, 134(3), pp. 320-330. San Diego, Calif.: Elsevier 10.1016/j.clim.2009.11.006

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Pemphigus vulgaris (PV) is a severe autoimmune bullous skin disease and is primarily associated with IgG against desmoglein 3 (dsg3), a desmosomal adhesion protein. In light of the recent association of autoreactive T helper (Th) 2 cells with active PV, the present study sought to relate the occurrence of Th2-regulated dsg3-specific autoantibody subtypes, i.e. IgE and IgG4, in 93 well-characterized PV patients. Patients with acute onset PV (n=37) showed the highest concentrations of serum IgE and IgG4 autoantibodies, which were significantly lower in PV patients in remission (n=14). Furthermore, there was a strong correlation between dsg3-reactive IgE and IgG4 in acute onset, but not in chronic active (n=42) or remittent patients. Additionally, intercellular IgE deposits were detected in the epidermis of acute onset PV. Thus, dsg3-specific IgE and IgG4 autoantibodies are related to acute onset disease which provides additional support to the concept that PV is a Th2-driven autoimmune disorder.

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