Marta Gamissans, Clara Muntaner-Virgili, Rosa-Maria Penín, Joaquim Marcoval
{"title":"Characteristics of Spontaneous Regression in Merkel Cell Carcinoma: A Retrospective Analysis of Patient and Tumor Features.","authors":"Marta Gamissans, Clara Muntaner-Virgili, Rosa-Maria Penín, Joaquim Marcoval","doi":"10.1093/ced/llae508","DOIUrl":"https://doi.org/10.1093/ced/llae508","url":null,"abstract":"<p><p>Merkel cell carcinoma (MCC) is an aggressive cutaneous neuroendocrine neoplasia. MCC complete spontaneous regression (CRS) has been linked to good prognosis. We conducted a retrospective review of a single-center cohort of MCC patients to analyze the characteristics of patients and tumors that exhibited regression. The calculated incidence of regressión was 4.5% (5/112). Median age at diagnosis was 69 years (RIQ 31), tumors were primarily located on the head and neck and patients had low rates of immunosuppression. After a median following-up of 4 years none of the patients showed recurrence or distant metastases. Merkel cell polyomavirus (MCPyV) positivity was associated with regression (P=0.045). Despite limited understanding of regression mechanisms, biopsy-induced immune responses linked with host and tumor factors such as age,immunosuppression or MCPyV positivity may play an important role. More research in this field is crucial for the comprehension of this rare phenomenon.</p>","PeriodicalId":10324,"journal":{"name":"Clinical and Experimental Dermatology","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142823619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Acquired autoinflammatory disorders: A dermatologist's perspective.","authors":"Anuradha Bishnoi, Apoorva Sharma, Narayanan Baskaran, Hitaishi Mehta, Debajyoti Chatterjee, Keshavamurthy Vinay","doi":"10.1093/ced/llae544","DOIUrl":"https://doi.org/10.1093/ced/llae544","url":null,"abstract":"<p><p>Autoinflammatory disorders are characterized by dysregulated and disproportionately heightened response of innate immune system (IIS) to molecular patterns associated with damage and pathogens/microbes (DAMPs/PAMPs) with crucial role played by neutrophils and macrophages in disease pathogenesis. They closely resemble connective tissue diseases (CTDs). However, anti-nuclear antibodies (ANA), typically considered a marker of CTDs, are negative in autoinflammatory disorders. Many autoinflammatory disorders are monogenic and arise due to inherited genetic mutations resulting in autoinflammation. This is especially true for disorders presenting in childhood or early adulthood. However, with advent of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, the genetic spectrum of these disorders has expanded, especially in adult population, emphasizing that these mutations could either be inherited or acquired later during one's lifetime. Additionally, many of the acquired autoinflammatory disorders, for example, adult-onset Still's disease (AOSD), and Schnitzler syndrome have a multifactorial pathogenesis and are typically polygenic. Many new disorders are being described in this category, and majority of them have prominent cutaneous manifestations, either at onset or during the course of disease, which are particularly important from diagnostic point of view. In this review, we discuss the cutaneous findings of a few acquired autoinflammatory disorders, with specific focus on AOSD, VEXAS syndrome, Schnitzler syndrome, Kikuchi-Fujimoto disease and hemophagolymphohistiocytosis (HLH).</p>","PeriodicalId":10324,"journal":{"name":"Clinical and Experimental Dermatology","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142821984","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lucas Boussingault, Yasaman Jazaeri, Dilara Sanak, Dana Bernardi, Anne-Laure Trépant, Elisa Cinotti, Linda Tognetti, Javiera Pérez-Anker, Jean Luc Perrot, Véronique Del Marmol, Mariano Suppa
{"title":"Multimodal skin imaging of a dermatofibrosarcoma protuberans using line-field confocal optical coherence tomography, ultra-high frequency ultrasound and reflectance confocal microscopy.","authors":"Lucas Boussingault, Yasaman Jazaeri, Dilara Sanak, Dana Bernardi, Anne-Laure Trépant, Elisa Cinotti, Linda Tognetti, Javiera Pérez-Anker, Jean Luc Perrot, Véronique Del Marmol, Mariano Suppa","doi":"10.1093/ced/llae543","DOIUrl":"https://doi.org/10.1093/ced/llae543","url":null,"abstract":"","PeriodicalId":10324,"journal":{"name":"Clinical and Experimental Dermatology","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142821985","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Li Jie Helena Yoo, Grace Hennessy, Odharnaith O'Brien, Jeremy Sargent, John Quinn, Siona Ní Raghallaigh
{"title":"Dermal mass with an annular figurate pattern in a patient with leukaemia.","authors":"Li Jie Helena Yoo, Grace Hennessy, Odharnaith O'Brien, Jeremy Sargent, John Quinn, Siona Ní Raghallaigh","doi":"10.1093/ced/llae538","DOIUrl":"https://doi.org/10.1093/ced/llae538","url":null,"abstract":"","PeriodicalId":10324,"journal":{"name":"Clinical and Experimental Dermatology","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142817043","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Usamah M Afzal, Faisal R Ali, Deemesh Oudit, Roxana Totorean, Patrick Shenjere, John T Lear
{"title":"Recurrent Morphoeic Basal Cell Carcinoma with Metastatic Progression in A 34-Year-Old Patient: A Case Report.","authors":"Usamah M Afzal, Faisal R Ali, Deemesh Oudit, Roxana Totorean, Patrick Shenjere, John T Lear","doi":"10.1093/ced/llae537","DOIUrl":"https://doi.org/10.1093/ced/llae537","url":null,"abstract":"","PeriodicalId":10324,"journal":{"name":"Clinical and Experimental Dermatology","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142817059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Luca Mastorino, Pedro Mendes-Bastos, Simone Ribero, Michela Ortoncelli
{"title":"The need for a shared definition of the atopic dermatitis \"Super Responder\" in the \"treat-to-target\" era.","authors":"Luca Mastorino, Pedro Mendes-Bastos, Simone Ribero, Michela Ortoncelli","doi":"10.1093/ced/llae542","DOIUrl":"https://doi.org/10.1093/ced/llae542","url":null,"abstract":"","PeriodicalId":10324,"journal":{"name":"Clinical and Experimental Dermatology","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142817143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Callum Riley, Sanaa Butt, Nomathamsanqa Mathe, Owen Cain, Ghazia Kaushal
{"title":"Bilateral lower limb ulceration in a patient with a malignancy.","authors":"Callum Riley, Sanaa Butt, Nomathamsanqa Mathe, Owen Cain, Ghazia Kaushal","doi":"10.1093/ced/llae536","DOIUrl":"https://doi.org/10.1093/ced/llae536","url":null,"abstract":"","PeriodicalId":10324,"journal":{"name":"Clinical and Experimental Dermatology","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142817456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ana Simon Gozalbo, Cristina Mauleon Fernandez, Raquel Carrascosa, Maria Angeles Martin Diaz, Pablo de le Cueva
{"title":"Refractory benign familial pemphigus successfully treated with dupilumab: a case series.","authors":"Ana Simon Gozalbo, Cristina Mauleon Fernandez, Raquel Carrascosa, Maria Angeles Martin Diaz, Pablo de le Cueva","doi":"10.1093/ced/llae507","DOIUrl":"https://doi.org/10.1093/ced/llae507","url":null,"abstract":"<p><p>Benign familial pemphigus (BFP) is a chronic autosomal dominant dermatosis characterized by the appearance of flaccid blisters which evolve to painful erythematous macerated plaques and erosions in intertriginous areas. While different medical therapies for BFP exist, current treatments are not consistently effective, and symptoms often cause decreased quality of life. We report a case series of three patients with refractory moderate-to severe BFP which have been successfully treated with dupilumab, describing clinical evolution within a follow-up period of 18 months. All patients reported a decrease in pruritus and improved skin findings with no adverse effects; although in one patient, increased dosing was required to achieve optimal control of disease. We also discuss previous reported cases of BFP treated with biologics. Dupilumab seems to be a useful and safe therapeutic option for refractory BFP cases.</p>","PeriodicalId":10324,"journal":{"name":"Clinical and Experimental Dermatology","volume":" ","pages":""},"PeriodicalIF":3.7,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142806346","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}