Yamaguchi Red Cross Hospital

Two distinct cases of giant epidermoid cysts in rare head and neck anatomical locations - one in the sublingual space and the other in the infratemporal fossa - are reported, highlighting the variability in presentation, imaging characteristics, and surgical management. Transoral excision of the cyst was selected for case 1 to minimize cosmetic concerns.For case 2, given the benign nature of the lesion and the patient's preference for a less invasive procedure, endoscopic marsupialization via a transnasal-transmaxillary approach was chosen. This method relieved symptoms, minimized surgical risks, and preserved quality of life, with no recurrence noted after three years. These cases underscore the importance of individualized treatment strategies for giant epidermoid cysts. While complete surgical excision remains the gold standard, minimally invasive options such as marsupialization may be appropriate in selected patients, especially when considering factors such as age, comorbidities, symptoms, and cosmetic outcomes.

To report an unusual case of combined Lie's types A and D of internal carotid artery (ICA) agenesis, diagnosed by magnetic resonance angiography (MRA). A 60-year-old woman with dizziness underwent cranial magnetic resonance imaging (MRI) and MRA of the intracranial region for the evaluation of brain and vascular lesions. The magnetic resonance machine was a 3.0-T scanner. MRI showed no abnormalities, except for multiple small white matter lesions. MRA showed that the left ICA was absent, except for the supraclinoid segment, and an anastomotic vessel was present between the paraclinoid segments of the bilateral ICAs, indicating Lie's type D ICA agenesis. The left posterior communicating artery (PCoA) was also present. Thus, there were also features of type A ICA agenesis. The anastomotic vessels between the bilateral ICAs and ipsilateral PCoA were relatively small in caliber. Lie's type D ICA agenesis usually does not communicate with the anterior and posterior circulations. We encountered a case of combined type D and type A ICA agenesis. To our knowledge, no similar case has been reported in the English literature. This is the second case of type D ICA agenesis with patent ipsilateral PCoA. We speculate that in case of type A ICA agenesis, when the development of the PCoA is insufficient to support collateral blood flow, an anastomotic vessel between bilateral ICAs may develop.

61 歳，男性。X－1 年 5 月より全身の瘙痒を自覚し，近医皮膚科で プレドニゾロン（PSL）10 mg/day の内服，クロベタゾールプロピオン酸エステル軟膏の外用，光線療法で治療されたが難治であった。徐々に症状の増悪を認めたため，血液検査を行ったところ抗 BP180 抗体の上昇を認め，X 年 2 月に前医総合病院皮膚科を紹介され受診した。蛍光抗体直接法で表皮真皮境界部に IgG と C3 の線状沈着がみられ，水疱性類天疱瘡（Bullous Pemphigoid：BP）の診断で高用量ステロイド内服（PSL 1 mg/kg/day），シクロスポリンやミゾリビンなどの免疫抑制剤の追加，ステロイドパルス療法，IVIG（免疫グロブリン静注）療法，血漿交換療法が施行され，入退院を繰り返しながら加療されたが，瘙痒と水疱の新生が持続し，精査・加療を目的に X 年 5 月に当院を紹介され受診した。初診時の BPDAI（Bullous Pemphigoid Disease Area Index）は 95 点，抗 BP180 抗体は 3830 U/ml と高値であった。高用量ステロイド内服に加え，アザチオプリンの追加，IVIG 療法を施行されたが瘙痒と水疱の新生が持続していた。既往症にアトピー性皮膚炎があり，Th2 炎症が強力に病態に関与している可能性を考慮されデュピルマブの投与が開始された。その後，瘙痒と水疱新生は速やかに消退し，病勢のコントロールが可能となり，以後 PSL の減量も可能となった。重篤な副作用がなく，アトピー性皮膚炎で使用経験のあるデュピルマブは BP の有力な治療選択肢の 1 つになりうると考えられる。

Background Previous studies have demonstrated dexamethasone (DEX)'s efficacy for coronavirus disease 2019 (COVID-19). In contrast, patients with residual lung field shading and symptoms after DEX treatment have been observed, and the efficacy of additional corticosteroids (AC) is unknown. Objectives We aimed to investigate the efficacy of AC in patients with COVID-19 with residual respiratory symptoms or who required oxygen therapy or invasive mechanical ventilation after DEX treatment. Methods This was a single-center, retrospective observational study including 261 patients with community-onset COVID-19, aged ≥ 18 years, admitted to our hospital between March 1, 2020, and May 31, 2021. Finally, 34 patients were included in the study who met all four of the following criteria: (1) required oxygen therapy or invasive ventilation, (2) were treated with DEX, (3) had residual shading on chest imaging after DEX treatment, or (4) had unimproved respiratory symptoms or oxygen saturation < 90%. We reviewed the medical records and clinical courses of 14 patients who received ACtherapy (AC group) and 20 patients who did not (non-additional corticosteroids or NC group). Results The 90-day mortality rate was 35.7% in the AC group and 25.0% in the NC group. There was no statistically significant difference between the two groups (p = 0.797). In addition, there was no difference between groups in the proportion of patients who required oxygen therapy at discharge (64% vs. 35%, p = 0.162). The time from the end of DEX therapy to discharge was significantly longer in the AC group (median 7.5 vs. 33 days, p = 0.019). Regarding serious adverse events, infection was statistically more common in the AC group than in the NC group (p = 0.005). Conclusions ACafter DEX treatment does not improve clinical outcomes and may prolong hospital stay.

Hereditary angioedema is a rare genetic disease characterized by repeated episodes of temporary organ swelling. Three types of HAE are known, of which HAE with normal C1 inactivator is difficult to be diagnosed due to its lack of laboratory abnormalities. Here, we describe a case of HAE with normal C1 inactivator and recurrent acute abdomen following low-dose estrogen-progestin therapy. Notably, genetic analysis by Sanger sequencing led to the identification of a recurrent heterozygous missense mutation c.988A>G (p.K330E) in the plasminogen (PLG) gene of the patient. Prophylactic tranexamic acid and on-demand selective bradykinin B2 receptor blockers are used to treat her symptoms.

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We investigated the efficacy of dutasteride add-on therapy to α-1 adrenoceptor antagonists in patients with benign prostate hyperplasia (BPH) in relation to the transitional zone index (TZI) and evaluated the cutoff value of TZI that predicted improvements of subjective symptoms at 6 months. Male BPH patients with prostate volume (PV) ≥ 30 mL receiving dutasteride 0.5 mg/d for 6 months as add-on therapy along with α-1 adrenoceptor antagonists were enrolled. PV, transitional zone volume (TZV), TZI, International Prostate Symptom Score (IPSS), and uroflowmetry parameters before and at 6 months with dutasteride add-on treatment were evaluated. Eighty-three patients were included. The changes of total IPSS, IPSS voiding subscore, IPSS quality of life score, and voided volume were significantly correlated with TZI. Among baseline parameters, TZV and TZI were significantly associated with the changes of total IPSS in univariate analysis, and only TZI remained as an independent predictive factor for improving total IPSS in multivariate analysis (odds ratio -8.3, P=.048). The cutoff point of TZI for predicting an improvement of the total IPSS by 6 points or more was 0.67 (area under the curve 0.71, sensitivity 0.62, specificity 0.79). A higher TZI was significantly associated with improvement of subjective symptoms but not uroflowmetric findings for BPH patients with 6 months of dutasteride add-on therapy along with α-1 adrenoceptor antagonists, and the predictive value of TZI for effective dutasteride add-on therapy was higher than 0.67. BPH patients using α-1 adrenoceptor antagonists with a TZI higher than 0.67 can be good candidates for add-on dutasteride therapy.