慢性皮膚粘膜カンジダ症  [in Japanese] CMCD: Chronic Mucocutaneous Candidiasis Disease  [in Japanese]

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Author(s)

    • 岡田 賢 OKADA Satoshi
    • 広島大学大学院医歯薬保健学研究院小児科学 Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences

Abstract

<p>  慢性皮膚粘膜カンジダ感染(CMC: <u>C</u>hronic <u>M</u>ucocutaneous <u>C</u>andidiasis)は,口腔粘膜,消化管,外性器,皮膚,爪を主要病変とし,難治性,反復性のカンジダ感染症を呈する"状態"を示す.カンジダに対する局所免疫には,ヘルパーT細胞の亜群であるTh17細胞と,それが産生するインターロイキン17(IL-17)が重要で,この免疫機構の破綻によりCMCを発症する.本項では,IL-17の障害を背景に発症する原発性免疫不全症である慢性皮膚粘膜カンジダ症(CMCD: CMC disease)と,症候性CMCについて概説する.CMCDと症候性CMCは,CMCを"主要な感染症状"とする原発性免疫不全症という共通の側面を持つ.他方で,CMCDではカンジダ以外の病原体に対する易感染性を原則的に認めないのに対して,症候性CMCでは他の病原体に対する易感染性や臓器症状の合併が認められる.過去の検討で,CMCDの原因としてIL-17シグナル伝達に直接関与する分子群の異常が報告されている.一方で,症候性CMCの原因としてTh17増殖分化に関与する分子群の異常が多く認められることから,両疾患はIL-17シグナル伝達に関与する分子群の異常により発症する遺伝性疾患であることが言える.本項では,CMCDと症候性CMCに着目し,臨床症状,分子病態を概説する.</p>

<p>  Chronic mucocutaneous candidiasis (CMC) is an infectious phenotype which is characterized by recurrent or persistent infections affecting the nails, skin, and oral and genital mucosae caused by Candida species. Th17 cells produce interleukin-17 (IL-17) and play an important role in host mucosal immunity to Candida. Recent studies revealed that an impairment of IL-17 immunity underlies development of CMC. CMC disease (CMCD) is a primary immunodeficiency disease which is defined as CMC in patients in the absence of other prominent clinical signs. However, this definition is not strict. Thus, CMCD is currently used to refer to patients presenting with CMC as the main clinical phenotype. As well as CMCD, CMC is a major infectious phenotype in syndromic CMC. However, patients with syndromic CMC also present other clinical and infectious manifestations in addition to CMC. The genetic defects which affect development and/or proliferation of Th17 cells have been identified in patients with syndromic CMC. In contrast, germline mutations in the genes which directly involved in IL-17 signaling have been identified in patients with CMCD. Here, we review current knowledge of IL-17-signaling defects and the genetic etiologies of CMCD and syndromic CMC.</p>

Journal

  • Japanese Journal of Clinical Immunology

    Japanese Journal of Clinical Immunology 40(2), 109-117, 2017

    The Japan Society for Clinical Immunology

Codes

  • NII Article ID (NAID)
    130005696887
  • Text Lang
    JPN
  • ISSN
    0911-4300
  • Data Source
    J-STAGE 
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