論文

17歳,女性.2ヵ月前より前胸部に弛緩性水疱,びらんを認め全身に拡大した.抗Dsg 1抗体価が高値であり,落葉状天疱瘡の疑いにて当科を受診した.皮膚病理組織所見,蛍光抗体法,ELISA法より落葉状天疱瘡と診断した.顔面や胸背部の広範囲にびらんを認め,Pemphigus Disease Area Index(PDAI)45点と重症であった.プレドニゾロン1mg/kg/日の内服を開始したが治療抵抗性であった.計2回のステロイドパルス療法,その後連続して血漿交換療法2回,大量IVIG療法を行い寛解が得られた.経過中,一見同程度に見える皮疹を定期的にPDAIで定量的に評価することが正確な治療効果判定に有用であった.若年発症例においても成人同様に必要十分な治療により寛解導入し,少量のステロイドでの寛解維持を目標とすべきであり,初期治療の選択が重要であると考えた.(著者抄録)

© The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Antimicrobial Chemotherapy. All rights reserved. Objectives: A carbapenem-resistant Enterobacter cloacae complex isolated in Tokyo, Japan, produced a carbapenemase that was detected by a Carba NP test and a modified carbapenem inactivation method, but none of the ‘Big Five’ carbapenemase genes was detected by PCR. This study aimed to identify the carbapenemase. Methods: Carbapenemase genes were screened by WGS. Next, we generated a recombinant plasmid in which the carbapenemase gene was inserted. We also extracted the carbapenemase gene-carrying plasmid from the E. cloacae complex. The effects of both plasmids on the antibiotic susceptibility of Escherichia coli were then tested. The carbapenemase gene-carrying plasmid in the E. cloacae complex was completely sequenced. Results: A novel carbapenemase gene, bla FRI-4 , encoded an amino acid sequence that was 93.2% identical to French imipenemase (FRI-1). E. coli transformed with bla FRI-4 showed reduced carbapenem susceptibility. A complete sequence of the bla FRI-4 -carrying 98 508 bp IncFII/IncR plasmid (pTMTA61661) showed that bla FRI-4 and the surrounding region (18.7 kb) were duplicated. Conclusions: The FRI-4-producing E. cloacae complex was isolated in Japan, whereas all other FRI variants have been found in Europe, suggesting that the spread of FRI carbapenemases is global.

© 2018 Japanese Dermatological Association The pathological mechanisms and immunological kinetics of drug-induced hypersensitivity syndrome (DIHS), including the relevance of interleukin (IL)-6, remain unclear. We report a case of drug adverse reaction that does not fulfill the diagnostic criteria of DIHS but mimics its characteristic features. Because the patient was under anti-IL-6 therapy at the onset, some symptoms typically seen in DIHS were absent, such as fever and leukocyte count abnormalities. However, the characteristic features of DIHS were clearly observed in the subsequent course, including the repeated recurrence of skin rash, prolonged liver dysfunction and reactivation of herpes viruses. This case suggested that IL-6 role at the onset is not a main factor to determine the subsequent pathomechanism of DIHS and attention should be paid to the preceding therapy for achieving accurate diagnosis.

© 2017 British Association of Dermatologists Electron beam therapy (EBT) is an established treatment for mycosis fungoides (MF), but evidence for the use of EBT in advanced cutaneous conditions is limited, and optimal scheduling of the regimen for such conditions remains unclear. We report the case of a 44-year-old woman diagnosed with MF with widespread cutaneous lesions, including multiple huge tumours in the craniofacial area. Low-dose total skin (TS)EBT and subsequent localized skin (LS)EBT achieved striking improvements in eruptions. Oral etretinate was also administered during therapy. Our experience implies that combined TSEBT and LSEBT may be worth attempting when a patient presents with both widespread lesions and prominent tumours, even when the tumours are extremely large.

© AlphaMed Press 2019 Extramammary Paget's disease (EMPD) is a rare cutaneous adenocarcinoma that clinicopathologically resembles breast cancer. The prognosis of metastatic EMPD is poor. Although several chemotherapies have been tried, the effects are temporary; better drugs and combinations are required. In the present study, we retrospectively analyze the efficacy and safety of combination of cisplatin, epirubicin, and paclitaxel in five metastatic EMPD cases. The efficacy was better than that for previously reported regimens: 80% partial responses, including two patients who were refractory to taxane- and/or platinum-based regimens. In terms of safety, four patients who were able to continue treatment exhibited acceptable tolerability. This is the first regimen to combine taxane and anthracycline. When treating breast cancer, anthracycline is regarded as the key cytotoxic agent, and anthracycline in combination with taxane constitutes a key chemotherapeutic regimen. Given our results, we speculate both drugs are critical chemotherapeutic agents for the treatment of metastatic EMPD.

© 2018 Japanese Society for Investigative Dermatology Background: There are limited treatment options for advanced non-melanoma skin cancers (NMSCs). To overcome this issue, we need to conduct clinical studies, however, there is a lack of information on how many patients with advanced NMSCs are treated annually in Japan. Objective: To investigate the actual number of advanced NMSC patients in Japan. Methods: A questionnaire survey was sent to 668 institutes to educe information on: 1) the numbers of patients with squamous cell carcinoma (SCC), extramammary Paget disease (EMPD), other skin origin carcinomas, and cutaneous angiosarcoma (CAS) admitted in 2016 and 2017; 2) the preferred first- and second-line chemotherapies; and 3) the anticipated for future development. Results: Questionnaires were returned from 383 (57.3%) institutes. They reported a total of 1765 patients over the 2 years. The annual number patients with SCC, EMPD, other skin carcinomas, and CAS was 323.5, 192.5, 126, and 240.5, respectively. We estimated the annual number of patients for all 668 institutes to be 1255.6. Current first- and second-line treatment for NMSCs were chemotherapy regimens, but immune checkpoint inhibitors were the most anticipated new drugs for SCC and CAS, while chemotherapy was still the most anticipated treatment for EMPD. Conclusion: Considering that during 2017, the number of deaths in Japan due to NMSC was reported to be 948, our estimated annual number of patients with NMSCs, 1255.6 seems to be an accurate estimation. As most of the treatment options for advanced NMSCs are outdated, the results of this study should be used to propose clinical studies.

＜文献概要＞76歳,女性.左第2趾原発の悪性黒色腫に対し,転移検索目的にFDG-PET/CTを施行したところ原発巣の他同側の左膝と左鼠径部にFDGの集積を認めた.足趾離断,膝病変切除および鼠径リンパ節郭清を行った.病理組織像で原発巣と左鼠径リンパ節は悪性黒色腫であったが,膝病変はCD68陽性泡沫状細胞が増生しており,悪性黒色腫の転移ではなく,色素性絨毛結節性滑膜炎であった.術後は悪性黒色腫に対しインターフェロンβ維持療法を行っていたが,肺転移を認めたためニボルマブ投与へ変更した.下垂体性副腎不全および認知症を発症したため治療を中止したが,3年3ヵ月時点で生存中である.FDG-PET/CTは悪性腫瘍の転移巣検索として広く使用されているが,FDG集積を認める病変として悪性腫瘍以外にも炎症疾患や感染症,良性腫瘍があり注意を要する.本症では術前の画像検査のみでは色素性絨毛結節性滑膜炎と診断できなかった.非典型的なFDG集積を認めた場合は組織診を含め追加検査が必要と考えた.

ダカルバジンを含む化学療法に不応となったIV期悪性黒色腫9例に海外で主に2nd lineの化学療法として使用されるカルボプラチン+パクリタキセル(CP)療法を施行した。1例が部分奏効(PR)、3例が安定(SD)、5例が進行(PD)し、無増悪生存期間(PFS)中央値は2.8ヵ月、全生存期間中央値は10.6ヵ月であった。忍容性は一般に良好で、44%の症例がCP療法により制御(PR+SD)され、PRとSD症例のPFSは7.0〜11.6ヵ月とPD症例よりも有意に長かったことから、本邦においてもCP療法はIV期悪性黒色腫に対する救済化学療法の1つになりうることが示唆された。(著者抄録)