Mechanisms of acantholysis in pemphigus foliaceus.
Pemphigus foliaceus (PF) is a dermatosis characterized by subcorneal vesicles and pathogenic IgG autoantibodies against desmoglein 1. PF IgG passively transferred into neonatal mice induces a blistering disease that duplicates the key findings of PF. In this study we have used this animal model to investigate the role of complement and IgG valence in triggering blister formation. In the passive transfer experiments, we found that PF IgG, as well as the F(ab')2 and Fab fragments, was capable of inducing the typical subcorneal blistering disease in both complement-deficient and complement-sufficient mice. Moreover, the disease activity in these mice correlated well with the dose of IgG or its proteolytic fragments injected in the animals. We conclude that neither complement activation nor IgG-mediated cell surface antigen crosslinking is required for the induction of acantholysis in the experimental PF model.
España
A
A
Department of Dermatology, Medical College of Wisconsin, Milwaukee 53226, USA.
Diaz
L A
LA
Mascaró
J M
JM
Giudice
G J
GJ
Fairley
J A
JA
Till
G O
GO
Liu
Z
Z
D000051:Acantholysis / Q000209:etiology* / Q000276:immunology* / Q000473:pathology; D000818:Animals; D000831:Animals, Newborn; D001323:Autoantibodies / Q000008:administration & dosage; D003167:Complement Activation; D003182:Complement C5 / Q000172:deficiency / Q000235:genetics; D003165:Complement System Proteins / Q000172:deficiency; D006801:Humans; D007116:Immunization, Passive; D007140:Immunoglobulin Fab Fragments / Q000008:administration & dosage; D007074:Immunoglobulin G / Q000008:administration & dosage; D051379:Mice; D008807:Mice, Inbred BALB C; D008817:Mice, Mutant Strains; D010392:Pemphigus / Q000150:complications* / Q000276:immunology* / Q000473:pathology
D001323:Autoantibodies; D003182:Complement C5; D007140:Immunoglobulin Fab Fragments; D007074:Immunoglobulin G; D003165:Complement System Proteins