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Cytokine Profiles Contribute to Understanding the Pathogenic Difference Between Good Syndrome and Oral Lichen Planus: Two Case Reports and Literature Review
Authors:Takashi Maehara  Masafumi Moriyama  Shintaro Kawano  Jun-Nosuke Hayashida  Sachiko Furukawa  Miho Ohta  Akihiko Tanaka  Masaki Yamauchi  Yukiko Ohyama  Tamotsu Kiyoshima  Seiji Nakamura
Institution:From the Section of Oral and Maxillofacial Oncology (TM, MM, SK, J-NH, SF, MO, AT, MY, SN); Section of Oral and Maxillofacial Surgery (YO); and Division of Maxillofacial Diagnostic and Surgical Sciences, Laboratory of Oral Pathology, Faculty of Dental Science, Kyushu University, Fukuoka, Japan (YK).
Abstract:We described and analyzed the pathogenic difference between Good syndrome (GS) and oral lichen planus (OLP) in oral mucosa.Good syndrome (GS) is a rare disease characterized by B and T cell immunodeficiency associated with hypogammaglobulinemia and thymoma. GS patients frequently develop oral lichenoid lesions with lymphocytic infiltration beneath the basal layer. Oral lichen planus (OLP) is a chronic inflammatory disease of the oral mucosa characterized by destruction of basal cells by Langerhans cells, macrophages, and T lymphocytes. Although the histological features of the lesions of both diseases are very similar, the pathogenesis of GS in the oral mucosa remains unknown. In this study, we thus investigated the expression of infiltrating lymphocyte subsets (CD3, CD20, CD4, and CD8) and T helper (Th) cytokines including interferon (IFN)-γ (Th1 type), interleukin (IL)-4 (Th2 type), IL-17 (Th17 type), and IL-10 (regulatory T cell type) by immunohistochemistry in buccal mucosa specimens from 2 GS patients compared with 15 OLP patients. All patients showed a predominance of CD3+ T cells over CD20+ B cells, and CD4+ Th cells over CD8+ cytotoxic T cells. This polarization was especially prominent in GS. IFN-γ and IL-10 were strongly detected in the infiltrating lymphocytes of all patients. However, IL-4 and IL-17 were detected in OLP patients only.These results suggest that the pathogenesis of GS is different from that of OLP. GS is a unique inflammatory disorder characterized by dysfunction of Th2 and Th17 immune reactions via abnormal T–B cell interaction.
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