label: url

en: 遅れて全身性エリテマトーデスを発症した高IgE症候群の1例

ja: 遅れて全身性エリテマトーデスを発症した高IgE症候群の1例

en: Hifuka No Rinsho

ja: 皮膚科の臨床


en: Exploration of Metabolite Biomarkers to Predict the Efficacy of Dupilumab Treatment for Atopic Dermatitis

en: Dupilumab (DUP) is the first biological agent used treating atopic dermatitis (AD). Notwithstanding its high cost, the type of patient group for which the drug is effective remains unclear. In this retrospective study, we aimed to identify novel and reliable biomarkers which can be measured before DUP administration and to predict the efficacy of DUP. Serum samples from 19 patients with AD treated with DUP were analysed by metabolome analysis using gas chromatography-mass spectrometry. Total 148 metabolites were detected, and the relative values of the metabolites were compared between the patient group that achieved 75% improvement in Eczema Area and Severity Index 16 weeks after administration of DUP (high responders: HR; n = 11) and that did not (low responders: LR; n = 8). The HR and LR groups had significant differences in the relative values of the eight metabolites (lactic acid, alanine, glyceric acid, fumaric acid, nonanoic acid, ribose, sorbitol, and ornithine), with ribose emerging as the best. Furthermore, we evaluated the serum concentrations of ribose and found that ribose may be a useful metabolite biomarker for predicting the efficacy of DUP in AD.

en: Disease Markers

ja: Disease Markers


en: Long-term Remission of Atopic Dermatitis after Discontinuation of Dupilumab

en: Acta Dermato-Venereologica

ja: Acta Dermato-Venereologica


en: A Case of Multiple Foreign Body Granulomas Developed on the Face and Extremities Caused by Injection with Hyaluronic Acid Fillers into Face

ja: 顔面へのヒアルロン酸製剤注入によって顔面と四肢に異物肉芽腫が多発した1例

en: A 69-year-old female with known eosinophilic fasciitis treated using 7.5 mg/day of prednisolone (PSL) was referred to our hospital for painful skin lesions following cosmetic treatment. One month before the first clinical visit, she received injections of hyaluronic acid fillers in the corners of her mouth at a cosmetic surgery clinic. Painful erythema and indurative lesions developed at the injection sites one week later, and she received hyaluronidase injections at the cosmetic surgery clinic. Subsequently, infiltrative erythema developed at non-injected sites on her face, left upper arm, and right thigh, and she was referred to our hospital. Histopathological examination of a biopsy specimen from indurated erythema on her right thigh revealed a foreign body granuloma in the subcutaneous tissue and the lower dermis. We administered 15 mg of PSL (total PSL dose :22.5 mg/day) and her symptoms gradually improved. She is currently being followed up with a tapered dose of PSL (12 mg/day). Most reported cases of foreign body granuloma after hyaluronic acid filler injections occurred at the injection sites. However, in this case, multiple cutaneous lesions of foreign body granuloma appeared at both the injected and non-injected sites. This is a rare case and suggested an association with the underlying eosinophilic fasciitis. Skin Research, 20 : 104-109, 2021

ja: 69歳，女性．基礎疾患に好酸球性筋膜炎があり，プレドニゾロン(以下 PSL)7.5mgを内服していた．初診の 1 ヶ月前に美容外科で両口角部にヒアルロン酸製剤の注入による施術を受けた．その 1 週間後に注入部位に疼痛を伴う紅斑，硬結が出現し，同科でヒアルロニダーゼを注入された．その後，顔面や四肢の非注入部位にも浸潤を伴う紅斑が出現したため，当科を紹介された．右大腿の硬結を伴う紅斑を生検したところ，病理組織学的に真皮深層から脂肪組織に異物肉芽腫の所見を認めた． PSL の投与量を 22.5mg/日に増量することによって症状が軽快したため，12 mg/日まで漸減して経過観察中である．国内外の文献で，ヒアルロン酸製剤注入による異物肉芽腫の報告はあるものの，ほとんどが注入部位に一致していた．自験例は注入部位以外に異物肉芽腫が多発性に出現した稀な症例であり，基礎疾患である好酸球性筋膜炎との関連も示唆される． (皮膚の科学，20 : 104-109, 2021)

en: Skin Research

ja: 皮膚の科学


en: Localized Cutaneous Nodular Amyloidosis with Different Clinical Features in the Epigastric Region and the Vulva

ja: 心窩部と外陰部に異なる皮膚症状を呈した皮膚限局性結節性アミロイドーシスの1例

en: A 62-year-old female was referred to our hospital for examination of a skin lesion on her left epigastric region, which developed 6 years before her first visit to our hospital. Orange-yellow or red papules aggregated and fused to form a slightly hard, shiny plaque on her left epigastric region. Furthermore, soft, partly wrinkled, and skin-colored or pink nodules developed on her left vulva 2 years before her visit. Histopathological examination of both skin lesions revealed amyloid deposition derived from immunoglobulin light chain kappa. Around the same time, she visited the department of urology for pain in her genital area and was diagnosed with bladder amyloidosis. Amyloid deposition was confirmed in her skin and bladder, but she was diagnosed with localized amyloidosis based on the amyloid-deposited organs, laboratory findings, and clinical course. The skin lesions on her epigastric region and vulva exhibited different clinical features, but both lesions were diagnosed as localized cutaneous nodular amyloidosis due to the histopathological findings. According to previous reports, localized cutaneous nodular amyloidosis exhibits many clinical features, and it may be overlooked in daily medical practice. Skin Research, 20 : 299-308, 2021

ja: 62歳，女性． 6 年前に自覚した心窩部左側の皮疹に対する精査目的で当科を紹介された．初診時，心窩部左側に橙黄色から紅色の丘疹が集簇・融合し，光沢を伴う弾性やや硬の局面を形成していた． また， 2 年前から外陰部左側に常色から淡紅色で一部に皺を伴う弾性軟の結節を認めていた．両者は病理組織学的に免疫グロブリン軽鎖(κ 型)由来のアミロイド沈着を認めた．同時期に，陰部の疼痛のために泌尿器科を受診し，膀胱アミロイドーシスと診断された．皮膚と膀胱の 2 臓器にアミロイドの沈着を認めたが，沈着臓器，検査所見，経過より総合的に限局性アミロイドーシスと判断した．心窩部と外陰部の皮膚症状は異なる臨床像を呈していたが，病理組織学所見よりいずれも皮膚限局性結節性アミロイドーシスと診断した．過去の報告を検討したところ，皮膚限局性結節性アミロイドーシスの臨床像は多彩であり，日常診療で見逃されている可能性があると考えた． (皮膚の科学，20 : 299-308, 2021)


en: 広範な血栓性静脈炎を呈し腸管ベーチェット病が疑われた1例

ja: 広範な血栓性静脈炎を呈し腸管ベーチェット病が疑われた1例


en: Three Cases of Generalized Pustular Psoriasis Successfully Treated with an Infliximab Biosimilar

ja: インフリキシマブBS(バイオ後続品)が有効であった膿疱性乾癬の3例

en: Case 1 is a 54-year-old female. Erythema appeared on her right upper arm and left wrist 10 days before the first visit. As the erythema had spread to her entire body and pustules had appeared, she was referred to our hospital. Her drug history raised suspicions of acute generalized exanthematous pustulosis, but systemic corticosteroid therapy was ineffective. Her poor response to corticosteroid therapy and the finding of an IL36RN gene mutation in a genetic test led to a decision to treat her for generalized pustular psoriasis. Her symptoms were improved after treatment with an infliximab biosimilar. Case 2 is a 36-year-old female. She developed psoriasis vulgaris at about age 20, and she had been treated by a local dermatologist since then. Erythema with small pustules appeared 10 months before her first visit, and she had started taking apremilast 5 months before that visit. Her symptoms were not improved, and she was referred to our hospital. She was given a diagnosis of generalized pustular psoriasis and treated with granulocyte and monocyte adsorptions plus an infliximab biosimilar, and her symptoms were improved. Case 3 is a 66-year-old man. At about age 15, he developed erythema and scales on his head. At about age 33, he developed a fever, and erythema with pustules appeared on his trunk. He had a diagnosis of generalized pustular psoriasis by a local dermatologist. He started taking etretinate, and his symptoms were improved. However, 1 month before the first visit, his erythema with pustules recurred and he was referred to our hospital. His symptoms improved after treatment with an infliximab biosimilar. Infliximab biosimilar drugs are approved for psoriasis by extrapolated drug reaction, and their efficacy and safety for generalized pustular psoriasis in Japan are unclear. In our hospital, an infliximab biosimilar was effective in all three cases, although the combination of an infliximab biosimilar with granulocyte and monocyte adsorptions or etretinate may contribute to that results. Skin Research, 19 :190-196, 2020

ja: 症例 1 は54歳，女性．初診10日前に右上腕，左手首に紅斑が出現した．全身に拡大し膿疱も出現したため，当科を紹介受診した．薬歴より急性汎発性発疹性膿疱症を疑い，ステロイド全身投与を行ったが無効であった．ステロイド治療の反応が乏しい点と，遺伝子検査で IL36RN 遺伝子の変異が判明した点より，膿疱性乾癬として加療する方針に変更した．インフリキシマブ BS(バイオ後続品)の投与で症状は軽快した．症例 2 は36歳，女性．20歳頃に尋常性乾癬を発症し，近医皮膚科で加療されていた．初診10ヶ月前に小膿疱が出現し， 5 ヶ月前にアプレミラストの内服を開始したが，症状の改善を認めず当科を紹介受診した．膿疱性乾癬の診断で，顆粒球吸着療法とインフリキシマブ BS の投与により症状は軽快した．症例 3 は66歳，男性．15歳頃に頭部の紅斑，鱗屑を自覚した．33歳頃に発熱と体幹に膿疱を伴う紅斑が出現し，近医皮膚科で膿疱性乾癬と診断されたが，エトレチナートの内服で症状は改善した．初診 1 ヶ月前より膿疱を伴う紅斑が再燃し，当科に紹介受診した．エトレチナートとインフリキシマブ BS の投与で症状は軽快した．インフリキシマブ BS は効果の外挿により乾癬への適応を取得しており，本邦での実臨床における膿疱性乾癬に対する有効性，安全性は不明である．顆粒球吸着療法やエトレチナートとの併用が寄与した可能性もあるが，当院ではすべての症例でインフリキシマブ BS が有効であった． (皮膚の科学，19 : 190-196, 2020)