Bullous pemphigoid sera induce bullous-pemphigoid-like lesions in neonatal mice pretreated with a limited dose of ultraviolet B irradiation.
The role of bullous pemphigoid (BP) autoantibodies (Abs) in the pathogenesis of BP is unclear. Lack of a confirmed experimental animal model prevents studies of the pathogenetic role of BP Abs. We hypothesized that some alterations of BP antigens (BP Ags) will be necessary for causative binding of BP Abs to the Ags. To cause an artificial and reproducible alteration, neonatal mice were irradiated with ultraviolet B (UVB). After the irradiation of 600 mJ/cm2 of UVB, BP serum was intraperitoneally injected. When the BP sera, which recognized only the 230-kD BP Ag, or both 230- and 180-kD BP Ags, were transferred into the UVB-treated mice, erosions and vesicles appeared in 14-78% of the animals. Histopathological examination revealed subepidermal blister formation in the mice treated with UVB and BP sera. Electron microscopy demonstrated that the separation occurred at the level of the lamina lucida. Human IgG and C3 were deposited at the dermal-epidermal junction. Control animals to which healthy sera were injected after the same dose of UVB irradiation showed degeneration of the upper epidermis but no apparent dermal-epidermal cleft formation histopathologically. These results suggest that BP antibodies play a pathogenetic role in vivo. This animal model can contribute to a study of the pathogenesis of BP.
Mitsuhashi
Y
Y
Department of Dermatology, Hirosaki University School of Medicine, Japan.
Nakano
H
H
Murai
T
T
Ohta
T
T
Sawamura
D
D
Hanada
K
K
Hashimoto
I
I
D000818:Animals; D000831:Animals, Newborn; D001323:Autoantibodies / Q000502:physiology; D003176:Complement C3 / Q000032:analysis; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D051379:Mice; D008807:Mice, Inbred BALB C; D010391:Pemphigoid, Bullous / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D011829:Radiation Dosage; D012867:Skin / Q000276:immunology / Q000528:radiation effects / Q000648:ultrastructure; D014466:Ultraviolet Rays
D001323:Autoantibodies; D003176:Complement C3; D007074:Immunoglobulin G