Table of Content


Autumn 2020, Vol. 28 No. 3

Hong Kong J. Dermatol. Venereol. (2020) 28, 100-109

Review Article

Spectrum of IgG4-related skin disease and differential diagnoses


Y Tokura 戶倉新樹


Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is characterised by high levels of circulating IgG4 and tissue infiltration of IgG4+ plasma cells. IgG4-RD exhibits a distinctive fibro-inflammatory change involving multiple organs, such as pancreas, and salivary and lacrimal glands. The skin manifestations of IgG4-RD, IgG4-related skin disease (IgG4-RSD) may stem from not only direct infiltration of plasma cells but also IgG4-mediated inflammation and can be categorised into seven subtypes: (1) cutaneous plasmacytosis, (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia, (3) Mikulicz's disease, (4) psoriasis-like eruption, (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinaemic purpura and urticarial vasculitis, and (7) ischaemic digit. It is considered that the subtypes (1)-(3) are induced by direct infiltration of IgG4+ plasma cells, while the other (4)-(7) types are caused by secondary mechanisms.

第四型免疫球蛋白G相關疾病的特徵是高血液水平的第四型免疫球蛋白G和第四型免疫球蛋白G陽性漿細胞的組織浸潤。第四型免疫球蛋白G相關疾病表現出獨特的纖維炎性改變,常涉及的多個器官,包括胰臟、唾液腺和淚腺。第四型免疫球蛋白G相關疾病及第四型免疫球蛋白G相關皮膚病的皮膚表現,不僅源於漿細胞的直接浸潤,還與第四型免疫球蛋白G介導的炎症有關,共可分為七類亞型:(1)皮膚漿細胞增多;(2 )假性淋巴瘤和血管淋巴樣增生伴嗜酸細胞增多症;(3)米庫利茲氏病;(4)銀屑病樣皮疹;(5)未明確的斑丘疹或紅斑性皮疹;(6)高球蛋白血症性紫癜和蕁麻疹性血管炎以及(7)缺血性手指腳趾。認為亞型(1)-(3)是由第四型免疫球蛋白G陽性漿細胞的直接浸潤引致的,而其他(4)-(7)類型則是由繼發機制引起。

Keywords: Cutaneous plasmacytosis, IgG4-related disease, pseudolymphoma

關鍵詞: 皮膚漿細胞增多症、第四型免疫球蛋白G相關疾病、假性淋巴瘤