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Atypical pemphigus with immunoglobulin G autoantibodies against desmoglein 3 and desmocollin 3
Authors:Koji Kamiya  Yumi Aoyama  Chie Wakashima  Tomoko Kudo  Kimiko Nakajima  Shigetoshi Sano  Norito Ishii  Kwesi Teye  Takashi Hashimoto  Keiji Iwatsuki  Yoshiki Tokura
Affiliation:1. Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan;2. Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan;3. Department of Dermatology, Kawasaki Hospital, Kawasaki Medical School, Okayama, Japan;4. Department of Dermatology, Kochi Medical School, Kochi University, Nankoku, Japan;5. Department of Dermatology, Kurume University School of Medicine and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan
Abstract: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.
Keywords:desmocollin 3  desmoglein 3  immunoglobulin G autoantibodies  immunostaining  pemphigus
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