Atypical pemphigus with immunoglobulin G autoantibodies against desmoglein 3 and desmocollin 3.
In patients with pemphigus vulgaris (PV), pathogenic immunoglobulin (Ig)G antibodies are most commonly directed against desmoglein 3 (Dsg3). It has recently been reported, however, that IgG anti-desmocollin 3 (Dsc3) antibodies are detected in some cases of pemphigus with or without IgG anti-Dsg3 antibodies. We present a case of pemphigus with IgG antibodies against Dsg3 and Dsc3. Subsequent studies showed that the cell surface distribution pattern of Dsc3 but not Dsg3 was altered, suggesting that suprabasal acantholytic blisters were induced by IgG anti-Dsc3 antibodies rather than IgG anti-Dsg3 antibodies. Our case suggests that anti-Dsc3 antibodies may be pathogenic in cases positive for the dual cadherin autoantibodies.
Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan.|Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Koji
K
Kamiya
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.|Department of Dermatology, Kawasaki Hospital, Kawasaki Medical School, Okayama, Japan.
Yumi
Y
Aoyama
Department of Dermatology, Kochi Medical School, Kochi University, Nankoku, Japan.
Chie
C
Wakashima
Department of Dermatology, Kochi Medical School, Kochi University, Nankoku, Japan.
Tomoko
T
Kudo
Department of Dermatology, Kochi Medical School, Kochi University, Nankoku, Japan.
Kimiko
K
Nakajima
Department of Dermatology, Kochi Medical School, Kochi University, Nankoku, Japan.
Shigetoshi
S
Sano
Department of Dermatology, Kurume University School of Medicine and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.
Norito
N
Ishii
Department of Dermatology, Kurume University School of Medicine and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.
Kwesi
K
Teye
Department of Dermatology, Kurume University School of Medicine and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.
Takashi
T
Hashimoto
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Keiji
K
Iwatsuki
Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Yoshiki
Y
Tokura
D001323:Autoantibodies / Q000032:analysis* / Q000276:immunology; D051187:Desmocollins / Q000097:blood / Q000276:immunology*; D051184:Desmoglein 3 / Q000097:blood / Q000276:immunology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis* / Q000276:immunology; D008856:Microscopy, Fluorescence; D008875:Middle Aged; D009061:Mouth Mucosa / Q000473:pathology; D010392:Pemphigus / Q000276:immunology* / Q000473:pathology
D001323:Autoantibodies; C490356:DSC3 protein, human; C071894:DSG3 protein, human; D051187:Desmocollins; D051184:Desmoglein 3; D007074:Immunoglobulin G
desmocollin 3; desmoglein 3; immunoglobulin G autoantibodies; immunostaining; pemphigus