Journal of Dermatology最新文献

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First reported case of thymoma-associated multiorgan autoimmunity induced by COVID-19 COVID-19诱发胸腺瘤相关多器官自身免疫的首例报告。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-26 DOI: 10.1111/1346-8138.17519
Rie Hyobu, Miho Mori, Tatsuo Maeda, Kazuki Fujimori, Yukako Shimai, Makiko Naito, Masayuki Masuda, Tatsuo Ohira, Norihiko Ikeda, Yukari Okubo, Kazutoshi Harada
{"title":"First reported case of thymoma-associated multiorgan autoimmunity induced by COVID-19","authors":"Rie Hyobu,&nbsp;Miho Mori,&nbsp;Tatsuo Maeda,&nbsp;Kazuki Fujimori,&nbsp;Yukako Shimai,&nbsp;Makiko Naito,&nbsp;Masayuki Masuda,&nbsp;Tatsuo Ohira,&nbsp;Norihiko Ikeda,&nbsp;Yukari Okubo,&nbsp;Kazutoshi Harada","doi":"10.1111/1346-8138.17519","DOIUrl":"10.1111/1346-8138.17519","url":null,"abstract":"<p>Thymoma-associated multiorgan autoimmunity (TAMA) presents with skin symptoms similar to those of graft-versus-host disease (GVHD), liver dysfunction, and enteritis, in the absence of a history of hematopoietic stem cell or bone marrow transplantation. TAMA is a type of paraneoplastic syndrome associated with thymoma. Its etiology is unclear but is thought to be a result of breakdown of immune tolerance. Histopathologically, TAMA is characterized by epidermal acanthosis with parakeratosis, individual cell keratinization, liquefaction degeneration, and intraepidermal infiltration of CD8-positive lymphocytes. A 64-year-old female patient with a history of myasthenia gravis and thymoma treated with prednisolone (10 mg/day) and cyclosporine (150 mg/day) experienced erythema on her trunk after coronavirus disease 2019 (COVID-19) onset. A psoriatic drug eruption was suspected and the possible causative drug was discontinued, but the skin rash failed to improve. A skin biopsy demonstrated GVHD-like histopathological findings. Diarrhea, abdominal pain, and duodenal perforation occurred concurrently, leading to the diagnosis of TAMA. Thereafter, the patient continued prednisolone and cyclosporine in the same doses as the TAMA treatment and added topical steroids. During the disease course, candida fungemia and cytomegalovirus infection developed, resulting in the patient's death. The TAMA was considered to have been caused by the release of inflammatory cytokines, autoreactive T cell activation, and regulatory T cell dysfunction induced by COVID-19.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"51 12","pages":"1674-1678"},"PeriodicalIF":2.9,"publicationDate":"2024-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11624154/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142515614","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The continuous increase in the number of newly medically attended cases of herpes zoster did not accelerate during the coronavirus disease 2019 pandemic in Kanagawa, Japan 在日本神奈川县 2019 年冠状病毒病大流行期间,带状疱疹新就诊病例的持续增长并未加快。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-22 DOI: 10.1111/1346-8138.17513
Yu Sakamoto, Ryo Watanabe
{"title":"The continuous increase in the number of newly medically attended cases of herpes zoster did not accelerate during the coronavirus disease 2019 pandemic in Kanagawa, Japan","authors":"Yu Sakamoto,&nbsp;Ryo Watanabe","doi":"10.1111/1346-8138.17513","DOIUrl":"10.1111/1346-8138.17513","url":null,"abstract":"<p>Possible changes in the epidemiology of herpes zoster (HZ) since the emergence of the coronavirus disease 2019 (COVID-19) pandemic is an unfolding topic. The number of newly medically attended cases (NMAC) of HZ during the pandemic in Kanagawa Prefecture, Japan, was quantitatively investigated using a claims database. The study period was from April 2017 to March 2022. The number of NMAC of HZ showed a continuously increasing trend throughout the study period, which could be attributed to population aging. Autoregressive integrated moving average modeling and forecasting revealed that the increase in the actual number of NMAC of HZ since the start of the pandemic was generally similar to that predicted based on a model fitted using data from the pre-pandemic period, but at a slightly reduced pace. This slowdown suggests that refraining from medical visits during the pandemic affected HZ surveillance.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 2","pages":"367-372"},"PeriodicalIF":2.9,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
BRAF/MEK inhibitor-induced tumor lysis syndrome in a patient with malignant melanoma 一名恶性黑色素瘤患者因 BRAF/MEK 抑制剂引发的肿瘤溶解综合征。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-22 DOI: 10.1111/1346-8138.17507
Shintaro Maeda, Katsuya Imazaki, Kyoko Shimizu, Kyosuke Oishi, Yasuhito Hamaguchi, Takashi Matsushita
{"title":"BRAF/MEK inhibitor-induced tumor lysis syndrome in a patient with malignant melanoma","authors":"Shintaro Maeda,&nbsp;Katsuya Imazaki,&nbsp;Kyoko Shimizu,&nbsp;Kyosuke Oishi,&nbsp;Yasuhito Hamaguchi,&nbsp;Takashi Matsushita","doi":"10.1111/1346-8138.17507","DOIUrl":"10.1111/1346-8138.17507","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 3","pages":"e242-e243"},"PeriodicalIF":2.9,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142515611","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Circulating CD31 and resistin levels reflect different stages of coronary atherosclerosis in patients with psoriasis 循环 CD31 和抵抗素水平反映了银屑病患者冠状动脉粥样硬化的不同阶段。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-22 DOI: 10.1111/1346-8138.17450
Trang Nguyen-Mai Huynh, Fumikazu Yamazaki, Robert J. Konrad, Yumiko Nishikawa, Akihiro Tanaka, Yonsu Son, Yoshio Ozaki, Kazuya Takehana, Hideaki Tanizaki
{"title":"Circulating CD31 and resistin levels reflect different stages of coronary atherosclerosis in patients with psoriasis","authors":"Trang Nguyen-Mai Huynh,&nbsp;Fumikazu Yamazaki,&nbsp;Robert J. Konrad,&nbsp;Yumiko Nishikawa,&nbsp;Akihiro Tanaka,&nbsp;Yonsu Son,&nbsp;Yoshio Ozaki,&nbsp;Kazuya Takehana,&nbsp;Hideaki Tanizaki","doi":"10.1111/1346-8138.17450","DOIUrl":"10.1111/1346-8138.17450","url":null,"abstract":"<p>Psoriasis is a skin disease with a complicated pathophysiology that includes an extensive inflammatory cytokine network. Nevertheless, the relationship between psoriasis severity, cytokine levels, and coronary artery atherosclerosis remains poorly understood. Our aim was to find serum markers as potential candidates for cardiovascular disease (CVD) risk monitoring in patients with psoriasis. Therefore, we examined coronary artery atherosclerosis via coronary computed tomography angiography (CCTA), serum cytokine levels via multiple immunoassays, and the patients' psoriasis state. Our findings reveal for the first time that the mainstream psoriasis cytokines interleukin 17A (IL-17A), IL-19, and IL-36 in the sera of Japanese patients with psoriasis showed a linear regression association with the Psoriasis Area and Severity Index score. Furthermore, the serum level of IL-19 was remarkably correlated to T<sub>h2</sub>-related serum cytokines such as IL-4 and IL-17E. When we investigated potential markers to monitor CVD in patients with psoriasis, circulating cluster of differentiation 31 (CD31) and resistin, but not psoriasis-related cytokines, were expressed differently at each stage of coronary atherosclerosis by CCTA. CD31 and resistin levels rose dramatically in individuals with psoriasis vulgaris (PV) and noncalcified atherosclerosis. In contrast, CD31 was negatively correlated with the coronary artery calcification score (CACS) in patients with PV, whereas resistin was inversely correlated with CACS in patients with psoriatic arthritis. In conclusion, the axis of IL-17A, IL-19, and IL-36 remains associated with the severity of psoriasis during the chronic phase of the disease, regardless of the application of topical or systemic treatment. Monitoring the levels of these cytokines can accurately determine the severity of skin inflammation. Resistin and CD31 are linked to coronary artery lesions and might be good candidates for tracking the progression of coronary atherosclerosis in patients with psoriasis.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 1","pages":"67-78"},"PeriodicalIF":2.9,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A case of mucous membrane pemphigoid solely with IgA anti-BP230 antibodies detected by immunoblotting using normal human epidermal extracts 使用正常人表皮提取物通过免疫印迹法检测到 IgA 抗 BP230 抗体的粘膜丘疹病病例。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-22 DOI: 10.1111/1346-8138.17511
Satoko Minakawa, Yasushi Matsuzaki, Takashi Hashimoto, Norito Ishii, Daisuke Sawamura, Eijiro Akasaka
{"title":"A case of mucous membrane pemphigoid solely with IgA anti-BP230 antibodies detected by immunoblotting using normal human epidermal extracts","authors":"Satoko Minakawa,&nbsp;Yasushi Matsuzaki,&nbsp;Takashi Hashimoto,&nbsp;Norito Ishii,&nbsp;Daisuke Sawamura,&nbsp;Eijiro Akasaka","doi":"10.1111/1346-8138.17511","DOIUrl":"10.1111/1346-8138.17511","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 3","pages":"e246-e247"},"PeriodicalIF":2.9,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484762","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Biallelic pathogenic variants in the LSS gene cause congenital alopecia-cataract syndrome LSS 基因中的双叶致病变体会导致先天性脱发-白内障综合征。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-22 DOI: 10.1111/1346-8138.17514
Yusha Chen, Shengyu Xie, Jia Geng, Zhongtao Li, Yuan Yang, Sheng Wang
{"title":"Biallelic pathogenic variants in the LSS gene cause congenital alopecia-cataract syndrome","authors":"Yusha Chen,&nbsp;Shengyu Xie,&nbsp;Jia Geng,&nbsp;Zhongtao Li,&nbsp;Yuan Yang,&nbsp;Sheng Wang","doi":"10.1111/1346-8138.17514","DOIUrl":"10.1111/1346-8138.17514","url":null,"abstract":"<p>Biallelic variants in the <i>LSS</i> gene have been reported in individuals affected by alopecia-intellectual disability syndrome 4, cataract 44, hypotrichosis 14, and palmoplantar keratoderma-congenital alopecia syndrome type 2. The present report described a Chinese girl with congenital alopecia universalis, cataract, esotropia, and nystagmus caused by compound heterozygous variants of c.1025T&gt;G (p.Ile342Ser) and previously unreported c.1011G&gt;A (p.Pro337=) in the <i>LSS</i> gene. Minigene assay confirmed the synonymous variant Pro337= at the edge of exon 9 could produce a novel splice site, leading to a 46-bp insertion of the 5′ sequence of the intron 9, likely resulting in a frameshift effect. We consider that the clinical manifestations of this case represent a new type of <i>LSS-</i>related disease, namely congenital alopecia-cataract syndrome (CACS). Our data expand the phenotypic and genetic spectrum of <i>LSS</i>-related diseases.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 3","pages":"531-535"},"PeriodicalIF":2.9,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A case of crateriform verruca on the right sole without human papilloma virus detection 一例右足底火山口状疣,未检出人类乳头瘤病毒。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-20 DOI: 10.1111/1346-8138.17508
Yu Nishimura, Hiroyuki Goto, Azusa Ogita, Kazushi Anzawa, Akira Shimizu, Daisuke Tsuruta
{"title":"A case of crateriform verruca on the right sole without human papilloma virus detection","authors":"Yu Nishimura,&nbsp;Hiroyuki Goto,&nbsp;Azusa Ogita,&nbsp;Kazushi Anzawa,&nbsp;Akira Shimizu,&nbsp;Daisuke Tsuruta","doi":"10.1111/1346-8138.17508","DOIUrl":"10.1111/1346-8138.17508","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 3","pages":"e244-e245"},"PeriodicalIF":2.9,"publicationDate":"2024-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484761","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Oral administration of ruxolitinib in psoriasis vulgaris: A case report of plaque psoriasis accompanied by myelofibrosis secondary to polisitemia vera successfully treated with oral ruxolitinib 口服芦可利替尼治疗寻常型银屑病:口服芦可利替尼成功治疗斑块状银屑病伴继发性骨髓纤维化的病例报告。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-20 DOI: 10.1111/1346-8138.17510
Tubanur Çetinarslan, İsmet Aydoğdu, Aylin Türel Ermertcan
{"title":"Oral administration of ruxolitinib in psoriasis vulgaris: A case report of plaque psoriasis accompanied by myelofibrosis secondary to polisitemia vera successfully treated with oral ruxolitinib","authors":"Tubanur Çetinarslan,&nbsp;İsmet Aydoğdu,&nbsp;Aylin Türel Ermertcan","doi":"10.1111/1346-8138.17510","DOIUrl":"10.1111/1346-8138.17510","url":null,"abstract":"<p>Psoriasis is a chronic inflammatory skin disease characterized by keratinocyte hyperproliferation and immune cell infiltration. Various therapies have been discovered for psoriasis, including topical treatments, phototherapy, conventional systemic agents such as methotrexate, retinoids and ciclosporine, as well as biologics. Janus kinase/signal transducer and activator of transcription (JAK/STAT) pathway inhibitors targeting Tumor Necrosis Factor alpha (TNF-α), interleukin (IL)-23 and IL-17 can be effective in psoriasis. Ruxolitinib is a US Food and Drug Administration-approved first-generation Janus kinase inhibitor for polycythemia vera, myelofibrosis, and acute graft-versus-host disease. Ruxolitinib cream has been investigated in various dermatologic diseases, including atopic dermatitis, vitiligo, psoriasis, and alopecia areata. However, there is limited data on the efficacy of oral ruxolitinib in patients with psoriasis vulgaris. Here, we report a patient diagnosed with myelofibrosis coexisting with psoriasis vulgaris, successfully treated with oral ruxolitinib.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 1","pages":"179-182"},"PeriodicalIF":2.9,"publicationDate":"2024-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484768","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Serum interleukin-10 level is increased and correlated positively with disease severity in patients with dipeptidyl peptidase-4 inhibitor-related bullous pemphigoid 二肽基肽酶-4 抑制剂相关大疱性类天疱疮患者血清白细胞介素-10 水平升高,并与疾病严重程度呈正相关。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-20 DOI: 10.1111/1346-8138.17512
Shoya Suzuki, Masahiro Kamata, Hideaki Uchida, Teruo Shimizu, Yoshiki Okada, Makoto Ito, Ayu Watanabe, Itsumi Mizukawa, Shota Egawa, Chika Chijiwa, Azusa Hiura, Saki Fukaya, Kotaro Hayashi, Atsuko Fukuyasu, Takamitsu Tanaka, Takeko Ishikawa, Yayoi Tada
{"title":"Serum interleukin-10 level is increased and correlated positively with disease severity in patients with dipeptidyl peptidase-4 inhibitor-related bullous pemphigoid","authors":"Shoya Suzuki,&nbsp;Masahiro Kamata,&nbsp;Hideaki Uchida,&nbsp;Teruo Shimizu,&nbsp;Yoshiki Okada,&nbsp;Makoto Ito,&nbsp;Ayu Watanabe,&nbsp;Itsumi Mizukawa,&nbsp;Shota Egawa,&nbsp;Chika Chijiwa,&nbsp;Azusa Hiura,&nbsp;Saki Fukaya,&nbsp;Kotaro Hayashi,&nbsp;Atsuko Fukuyasu,&nbsp;Takamitsu Tanaka,&nbsp;Takeko Ishikawa,&nbsp;Yayoi Tada","doi":"10.1111/1346-8138.17512","DOIUrl":"10.1111/1346-8138.17512","url":null,"abstract":"<p>Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease with a linear deposit of autoantibodies at the epidermal basement membrane zone. It was reported that diabetes patients who took a dipeptidyl peptidase-4 inhibitor (DPP4i), an oral antidiabetic drug, had an increased incidence of BP. However, data on DPP4i-related BP are limited. In our study, we measured serum levels of various cytokines using LEGENDplex and assessed correlations of these serum levels with clinical severity and laboratory data. Serum samples obtained from BP patients who visited our hospital from June 2016 to February 2023 were collected in this study. Patients' background, characteristics, and clinical data were retrospectively collected from their charts. Serum samples from 27 patients with DPP4i-unrelated BP, 17 patients with DPP4i-related BP, and 13 healthy controls were analyzed. Patients with DPP4i-related BP had lower score of Bullous Pemphigoid Disease Area Index (BPDAI)-erythema/urticaria, lower number of circulating eosinophils, and lower titer of anti-BP180 antibody than patients with DPP4i-unrelated BP. The serum interleukin (IL)-6 level was significantly higher in patients with DPP4i-related BP than in healthy controls (<i>P</i> = 0.0037). The serum IL-10 level was significantly higher in patients with DPP4i-related BP than in patients with DPP4i-unrelated BP and in healthy controls (<i>P</i> = 0.0006, <i>P</i> = 0.0448), and was positively correlated with the BPDAI-blister/erosions score (<i>r</i> = 0.757, <i>P</i> = 0.001), BPDAI-erythema/urticaria score (<i>r</i> = 0.616, <i>P</i> = 0.013), and BPDAI-total score (<i>r</i> = 0.833, <i>P</i> &lt; 0.001) in patients with DPP4i-related BP. In conclusion, patients with DPP4i-related BP had increased serum levels of IL-6 and IL-10 compared with healthy controls and positive correlations between the serum IL-10 level and BPDAI scores reflecting clinical severity, indicating that the serum IL-10 level is a potential objective biomarker of disease severity in patients with DPP4i-related BP.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 1","pages":"122-131"},"PeriodicalIF":2.9,"publicationDate":"2024-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484770","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Postoperative continuous compression bandaging was a useful technique for improving pseudosyndactyly in recessive dystrophic epidermolysis bullosa patients 术后持续加压包扎是改善隐性萎缩性表皮松解症患者假性畸形的有效技术。
IF 2.9 3区 医学
Journal of Dermatology Pub Date : 2024-10-16 DOI: 10.1111/1346-8138.17509
Kosuke Mochida, Yukiyo Narita, Masahiro Amano
{"title":"Postoperative continuous compression bandaging was a useful technique for improving pseudosyndactyly in recessive dystrophic epidermolysis bullosa patients","authors":"Kosuke Mochida,&nbsp;Yukiyo Narita,&nbsp;Masahiro Amano","doi":"10.1111/1346-8138.17509","DOIUrl":"10.1111/1346-8138.17509","url":null,"abstract":"&lt;p&gt;Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic skin disorder characterized by variants in the &lt;i&gt;COL7A1&lt;/i&gt; gene, which encodes type VII collagen, essential for the basement membrane zone and the formation of anchoring fibrils. Because of the reduced or absent type VII collagen, blistering and subsequent scarring of the hands and feet lead to fusion of the digits accompanied by contractures and pseudosyndactyly. Repeated surgical intervention is often necessary to temporarily improve hand function and delay the recurrence of deformity.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;A 36-year-old woman with RDEB was referred to our department for pseudosyndactyly of the left hand, having undergone four surgeries previously, the latest 3 years earlier (Figure 1a,b). Owing to insufficient donor sites for skin grafts, artificial dermis was used for wound coverage. The operation began with removing the inelastic epidermis, which released the web and flexion contractures. The fingers were separated by blunt dissection to the web base, and flexion contractures were addressed with transverse volar incisions extending to the finger sides (Figure 1c,d). After exposing the dermis, artificial dermis grafts were applied to cover the palm and fingers followed by application of polymyxin B-soaked gauze to maintain a wet-to-dry environment and non-adherent dressing. A boxing-glove type dressing was used to maintain wrist dorsiflexion, metacarpophalangeal (MCP) and interphalangeal (IP) joint extension, and thumb abduction without Kirschner wires.&lt;/p&gt;&lt;p&gt;The first dressing change was on day 5 post-surgery, with the removal of the silicon film from the artificial dermis grafts. Non-adherent vaseline gauze (ADAPTIC&lt;sup&gt;®&lt;/sup&gt;; 3M) was used to cover the hands, and an alginate coating dressing (KALTOSTAT&lt;sup&gt;®&lt;/sup&gt;; Convatec) placed over it. Alginate dressing was also placed between the fingers to prevent web space obliteration and flexion contracture, with each finger fixed using a self-adhesive bandage for traction (Figure 1e–h). Continued dressing changes maintained a good condition, and although complete epithelialization took over 9 weeks (Figure 1i–l), the patient was satisfied with the outcome and improved hand use in daily activities. Three years and 8 months postoperatively, there is no adhesion of the fingers, and the patient has had a favorable clinical course (Figure 1m,n).&lt;/p&gt;&lt;p&gt;Most RDEB patients opt for hand surgeries to regain function. Treatments such as allogeneic fibroblast injections and skin substitutes show benefits but are not curative, and recurrence is unavoidable.&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; Box et al. reported that 71.4% of the survey respondents used pins during surgery and recommended wearing post-operative hand orthoses to preserve surgical results. However, recurrence occurred in approximately 50% of cases after 1 year, requiring additional procedure.&lt;span&gt;&lt;sup&gt;3&lt;/sup&gt;&lt;/span&gt; By utilizing postoperative compressive bandaging, the use of ","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"51 12","pages":"e414-e415"},"PeriodicalIF":2.9,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11624149/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142484769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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