IgG4‐related skin disease

British Journal of DermatologyVolume 171, Issue 5 p. 959-967 Review Article IgG4-related skin disease Y. Tokura, Corresponding Author Y. Tokura Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 Japan Correspondence Yoshiki Tokura. E-mail: tokura@hama-med.ac.jpSearch for more papers by this authorH. Yagi, H. Yagi Section of Dermatology, Shizuoka General Hospital, 4-27-1 Kita-Andou, Aoi-ku, Shizuoka, 420-8527 JapanSearch for more papers by this authorH. Yanaguchi, H. Yanaguchi Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 JapanSearch for more papers by this authorY. Majima, Y. Majima Section of Dermatology, Shizuoka General Hospital, 4-27-1 Kita-Andou, Aoi-ku, Shizuoka, 420-8527 JapanSearch for more papers by this authorA. Kasuya, A. Kasuya Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 JapanSearch for more papers by this authorT. Ito, T. Ito Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 JapanSearch for more papers by this authorM. Maekawa, M. Maekawa Laboratory Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 JapanSearch for more papers by this authorH. Hashizume, H. Hashizume Section of Dermatology, Shimada Municipal Hospital, 1200-5 Noda, Shimada, 427-8502 JapanSearch for more papers by this author Y. Tokura, Corresponding Author Y. Tokura Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 Japan Correspondence Yoshiki Tokura. E-mail: tokura@hama-med.ac.jpSearch for more papers by this authorH. Yagi, H. Yagi Section of Dermatology, Shizuoka General Hospital, 4-27-1 Kita-Andou, Aoi-ku, Shizuoka, 420-8527 JapanSearch for more papers by this authorH. Yanaguchi, H. Yanaguchi Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 JapanSearch for more papers by this authorY. Majima, Y. Majima Section of Dermatology, Shizuoka General Hospital, 4-27-1 Kita-Andou, Aoi-ku, Shizuoka, 420-8527 JapanSearch for more papers by this authorA. Kasuya, A. Kasuya Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 JapanSearch for more papers by this authorT. Ito, T. Ito Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 JapanSearch for more papers by this authorM. Maekawa, M. Maekawa Laboratory Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, 431-3192 JapanSearch for more papers by this authorH. Hashizume, H. Hashizume Section of Dermatology, Shimada Municipal Hospital, 1200-5 Noda, Shimada, 427-8502 JapanSearch for more papers by this author First published: 26 July 2014 https://doi.org/10.1111/bjd.13296Citations: 75 Funding sources: None. Conflicts of interest: None declared. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Summary IgG4-related disease (IgG4-RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4+ plasma cells. IgG4-RD exhibits a distinctive fibroinflammatory change involving multiple organs, such as the pancreas and salivary and lacrimal glands. The skin lesions of IgG4-RD have been poorly characterized and may stem not only from direct infiltration of plasma cells but also from IgG4-mediated inflammation. Based on the documented cases together with ours, we categorized the skin lesions into seven subtypes: (1) cutaneous plasmacytosis (multiple papulonodules or indurations on the trunk and proximal part of the limbs), (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia (plaques and papulonodules mainly on the periauricular, cheek and mandible regions), (3) Mikulicz disease (palpebral swelling, sicca syndrome and exophthalmos), (4) psoriasis-like eruption (strikingly mimicking psoriasis vulgaris), (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinaemic purpura (bilateral asymmetrical palpable purpuric lesions on the lower extremities) and urticarial vasculitis (prolonged urticarial lesions occasionally with purpura) and (7) ischaemic digit (Raynaud phenomenon and digital gangrene). It is considered that subtypes 1–3 are induced by direct infiltration of IgG4+ plasma cells, while the other types (4–7) are caused by secondary mechanisms. IgG4-related skin disease is defined as IgG4+ plasma-cell-infiltrating skin lesions that form plaques, nodules or tumours (types 1–3), but may manifest secondary lesions caused by IgG4+ plasma cells and/or IgG4 (types 4–7). Citing Literature Volume171, Issue5November 2014Pages 959-967 RelatedInformation