{"title":"A pediatric case of SAPHO syndrome without palmoplantar pustulosis.","authors":"Masato Ishikawa, Toshiyuki Yamamoto","doi":"10.1111/1346-8138.17646","DOIUrl":"https://doi.org/10.1111/1346-8138.17646","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143054305","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alopecia areata (AA) is a chronic, autoimmune skin disease characterized by non-scarring hair loss. Baricitinib, a Janus kinase inhibitor (JAKi), prevents hair loss and promotes hair regrowth by inhibiting the inflammatory Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling pathway involved in cytotoxic T cell responses targeting hair follicles. The introduction of JAKi has transformed treatment against severe AA. However, treatment responses to JAKi are highly variable among patients, and the predictors of responsiveness remain insufficiently elucidated. This study aimed to identify independent predictive factors for the efficacy of baricitinib in patients with severe AA using multivariate analyses. A retrospective study was conducted on 70 severe AA patients who started baricitinib treatment at Tohoku University Hospital between July 2022 and August 2023. The primary outcome was the percentage of patients achieving a Severity of Alopecia Tool (SALT) score of ≤20 after 9 months of baricitinib treatment. Multivariate analysis assessed potential predictors of baricitinib treatment responses, including AA type, sex, age, disease duration, history of atopic dermatitis, intravenous methylprednisolone pulse (IVMP) therapy, and Clinician-Reported Outcome (ClinRO) measures for eyebrows and eyelashes. Achievement of a SALT score of ≤20 and SALT score improvement rates were used as objective variables in the multivariate analyses. Among the 70 patients completing 9 months of baricitinib treatment, 41% achieved a SALT score of ≤20. Multivariate analyses identified several independent predictors for positive outcomes, including shorter disease duration (≤4 years), history of IVMP, therapy SALT score of ≤95 at baricitinib initiation, and female sex. Further, we found differential response patterns based on AA type and sex. Specifically, AA type significantly influenced treatment responses, with ophiasis alopecia (OA) associated with the poorest improvement rate. In summary, the response to baricitinib in AA is significantly influenced by sex, AA type, disease duration, history of IVMP, and pre-treatment SALT score.
{"title":"Predictive factors for treatment responses to baricitinib in severe alopecia areata: A retrospective, multivariate analysis of 70 cases from a single center.","authors":"Moyuka Wada-Irimada, Takehiro Takahashi, Mana Sekine, Toshiki Okazaki, Takuya Takahashi, Tomoko Chiba, Emi Yamazaki, Kosuke Shido, Toshiya Takahashi, Masato Mizuashi, Yoshihide Asano","doi":"10.1111/1346-8138.17641","DOIUrl":"https://doi.org/10.1111/1346-8138.17641","url":null,"abstract":"<p><p>Alopecia areata (AA) is a chronic, autoimmune skin disease characterized by non-scarring hair loss. Baricitinib, a Janus kinase inhibitor (JAKi), prevents hair loss and promotes hair regrowth by inhibiting the inflammatory Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling pathway involved in cytotoxic T cell responses targeting hair follicles. The introduction of JAKi has transformed treatment against severe AA. However, treatment responses to JAKi are highly variable among patients, and the predictors of responsiveness remain insufficiently elucidated. This study aimed to identify independent predictive factors for the efficacy of baricitinib in patients with severe AA using multivariate analyses. A retrospective study was conducted on 70 severe AA patients who started baricitinib treatment at Tohoku University Hospital between July 2022 and August 2023. The primary outcome was the percentage of patients achieving a Severity of Alopecia Tool (SALT) score of ≤20 after 9 months of baricitinib treatment. Multivariate analysis assessed potential predictors of baricitinib treatment responses, including AA type, sex, age, disease duration, history of atopic dermatitis, intravenous methylprednisolone pulse (IVMP) therapy, and Clinician-Reported Outcome (ClinRO) measures for eyebrows and eyelashes. Achievement of a SALT score of ≤20 and SALT score improvement rates were used as objective variables in the multivariate analyses. Among the 70 patients completing 9 months of baricitinib treatment, 41% achieved a SALT score of ≤20. Multivariate analyses identified several independent predictors for positive outcomes, including shorter disease duration (≤4 years), history of IVMP, therapy SALT score of ≤95 at baricitinib initiation, and female sex. Further, we found differential response patterns based on AA type and sex. Specifically, AA type significantly influenced treatment responses, with ophiasis alopecia (OA) associated with the poorest improvement rate. In summary, the response to baricitinib in AA is significantly influenced by sex, AA type, disease duration, history of IVMP, and pre-treatment SALT score.</p>","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143049378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sonja Dorfer, Michael Ablinger, Monika Wimmer, Julia I Hummel, Selma Ibrahimpašić, Anja Diem, Martin Laimer, Stefanie Gruner, Josefina Piñón Hofbauer, Christina Guttmann-Gruber, Ulrich Koller, Iris K Gratz, Johann W Bauer, Roland Zauner, Verena Wally
Recessive dystrophic epidermolysis bullosa (RDEB) is caused by mutations in COL7A1, leading to loss or dysfunction of type-VII collagen (C7), a protein essential for skin stability. Clinically, patients suffer from severe skin blistering, chronic or recurrent wounds, and scarring, which predispose to early onset of aggressive squamous cell carcinoma. Previous studies showed that RDEB-keratinocytes (RDEB-KC) express high levels of matrix-metalloproteinase 9 (MMP-9), a molecule known to play a crucial role in wound chronification if dysregulated. We investigated the potential of diacerein, a small molecule that interferes with the MMP-9 regulatory pathway, to improve wound healing in a 5-year old RDEB patient presenting with chronic, generalized skin involvement unresponsive to previous treatment approaches. Upon 4 weeks of topical therapy applied to the patient's back, parents reported a nearly complete wound closure and a significant increase in quality of life. We also provide evidence that diacerein treatment of patient keratinocytes results in a downregulation of MMP-9 expression, accompanied by a reduction in their ability to degrade a fibrinogen matrix. These data characterize diacerein as a potential candidate for improving wound healing in RDEB through its impact on inflammatory as well as epithelial cells.
{"title":"Repurposing diacerein for the treatment of chronic wounds in recessive-dystrophic epidermolysis bullosa patients by modulating matrix metalloproteinase-9 expression.","authors":"Sonja Dorfer, Michael Ablinger, Monika Wimmer, Julia I Hummel, Selma Ibrahimpašić, Anja Diem, Martin Laimer, Stefanie Gruner, Josefina Piñón Hofbauer, Christina Guttmann-Gruber, Ulrich Koller, Iris K Gratz, Johann W Bauer, Roland Zauner, Verena Wally","doi":"10.1111/1346-8138.17621","DOIUrl":"https://doi.org/10.1111/1346-8138.17621","url":null,"abstract":"<p><p>Recessive dystrophic epidermolysis bullosa (RDEB) is caused by mutations in COL7A1, leading to loss or dysfunction of type-VII collagen (C7), a protein essential for skin stability. Clinically, patients suffer from severe skin blistering, chronic or recurrent wounds, and scarring, which predispose to early onset of aggressive squamous cell carcinoma. Previous studies showed that RDEB-keratinocytes (RDEB-KC) express high levels of matrix-metalloproteinase 9 (MMP-9), a molecule known to play a crucial role in wound chronification if dysregulated. We investigated the potential of diacerein, a small molecule that interferes with the MMP-9 regulatory pathway, to improve wound healing in a 5-year old RDEB patient presenting with chronic, generalized skin involvement unresponsive to previous treatment approaches. Upon 4 weeks of topical therapy applied to the patient's back, parents reported a nearly complete wound closure and a significant increase in quality of life. We also provide evidence that diacerein treatment of patient keratinocytes results in a downregulation of MMP-9 expression, accompanied by a reduction in their ability to degrade a fibrinogen matrix. These data characterize diacerein as a potential candidate for improving wound healing in RDEB through its impact on inflammatory as well as epithelial cells.</p>","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143034345","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of localized scleroderma with variable response to excimer light therapy.","authors":"Kyoko Kimura, Takeshi Fukumoto, Akiharu Kubo","doi":"10.1111/1346-8138.17635","DOIUrl":"https://doi.org/10.1111/1346-8138.17635","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019166","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of psoriasiform drug eruption revealed by a drug-induced lymphocyte stimulation test.","authors":"Satoshi Takeuchi, Yasuko Shiomichi, Takeshi Nakahara","doi":"10.1111/1346-8138.17638","DOIUrl":"https://doi.org/10.1111/1346-8138.17638","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019208","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Japanese case of Papillon-Lefèvre syndrome diagnosed by pyogenic liver abscess.","authors":"Akari Terada, Takaya Murai, Eijiro Akasaka","doi":"10.1111/1346-8138.17631","DOIUrl":"https://doi.org/10.1111/1346-8138.17631","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Identification of key malodor components in onychomycosis: A pilot study.","authors":"Keiko Sakamoto, Masako Katsuyama, Chieko Okamura, Tetsuya Honda","doi":"10.1111/1346-8138.17633","DOIUrl":"https://doi.org/10.1111/1346-8138.17633","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019299","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vitiligo is a chronic autoimmune disorder that profoundly impacts patients' quality of life. Real-world data on vitiligo in Japan are limited. This descriptive, cross-sectional study used a claims database to evaluate vitiligo prevalence, patient demographics, treatments, and comorbidities in Japanese patients with vitiligo. Patients with claims for a vitiligo diagnosis in the JMDC database from January 2010 to December 2022 were included. Annual vitiligo prevalence, comorbidities, treatments, and medical facility visits were analyzed. Of 16 947 087 patients in the database during the 13-year analysis period, a total of 26 358 patients (0.16%, 95% confidence interval 0.15-0.16) had a diagnosis of vitiligo. The standardized prevalence of vitiligo by sex and age in Japan remained generally consistent between 2010 (0.051%) and 2022 (0.056%). Atopic dermatitis was the most prevalent comorbidity. Comorbid atopic dermatitis prevalence increased between 2010 (21.8%) and 2022 (34.0%), and was highest among children aged 5-9 years. Other common comorbidities in 2022 included hypertension (10.4%), dyslipidemia (8.0%), anxiety disorder (7.4%), and psoriasis (7.0%). Topical corticosteroids were the most commonly used treatment throughout the period analyzed. Between 2010 and 2022, topical corticosteroid use decreased from 75.1% to 66.9%, and the use of narrowband ultraviolet B procedures increased from 19.2% to 28.1%. Mean duration of care was 12.9 months (standard deviation 20.5 months) and the median total number of outpatient medical facility visits was 3.0 (interquartile range 1.0-12.0). Key limitations include age and occupational biases in the JMDC database and potential misclassification of comorbidities due to off-label treatment coding. Despite limitations in using a claims database, this study demonstrates consistent vitiligo prevalence in Japan, a high comorbidity burden, and evolving treatment patterns. Findings may guide clinical practice and treatment guidelines to improve management of vitiligo in Japanese patients.
{"title":"Clinical patterns of vitiligo in Japan: A descriptive study using the JMDC claims database.","authors":"Atsushi Tanemura, Yue Ma, Masayo Sakaki-Yumoto, Shintaro Hiro, Tomohiro Hirose, Tamio Suzuki","doi":"10.1111/1346-8138.17627","DOIUrl":"https://doi.org/10.1111/1346-8138.17627","url":null,"abstract":"<p><p>Vitiligo is a chronic autoimmune disorder that profoundly impacts patients' quality of life. Real-world data on vitiligo in Japan are limited. This descriptive, cross-sectional study used a claims database to evaluate vitiligo prevalence, patient demographics, treatments, and comorbidities in Japanese patients with vitiligo. Patients with claims for a vitiligo diagnosis in the JMDC database from January 2010 to December 2022 were included. Annual vitiligo prevalence, comorbidities, treatments, and medical facility visits were analyzed. Of 16 947 087 patients in the database during the 13-year analysis period, a total of 26 358 patients (0.16%, 95% confidence interval 0.15-0.16) had a diagnosis of vitiligo. The standardized prevalence of vitiligo by sex and age in Japan remained generally consistent between 2010 (0.051%) and 2022 (0.056%). Atopic dermatitis was the most prevalent comorbidity. Comorbid atopic dermatitis prevalence increased between 2010 (21.8%) and 2022 (34.0%), and was highest among children aged 5-9 years. Other common comorbidities in 2022 included hypertension (10.4%), dyslipidemia (8.0%), anxiety disorder (7.4%), and psoriasis (7.0%). Topical corticosteroids were the most commonly used treatment throughout the period analyzed. Between 2010 and 2022, topical corticosteroid use decreased from 75.1% to 66.9%, and the use of narrowband ultraviolet B procedures increased from 19.2% to 28.1%. Mean duration of care was 12.9 months (standard deviation 20.5 months) and the median total number of outpatient medical facility visits was 3.0 (interquartile range 1.0-12.0). Key limitations include age and occupational biases in the JMDC database and potential misclassification of comorbidities due to off-label treatment coding. Despite limitations in using a claims database, this study demonstrates consistent vitiligo prevalence in Japan, a high comorbidity burden, and evolving treatment patterns. Findings may guide clinical practice and treatment guidelines to improve management of vitiligo in Japanese patients.</p>","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}