The Journal of dermatology最新文献

Psoriasis is a chronic inflammatory skin condition associated with significant comorbidities that impact quality of life. Effective patient engagement through shared decision-making (SDM) is crucial for optimal management. This study aimed to evaluate the effectiveness of adding clinician-created educational videos in enhancing patient knowledge and engagement during SDM for psoriasis treatment. Forty-eight patients with moderate to severe psoriasis participated in this single-center study. After reading an educational pamphlet, patients took a knowledge assessment test. Subsequently, they watched an educational video and completed a second test using the same questions. Feedback questionnaires on the video and the SDM process were also administered. Paired t tests revealed that postpamphlet plus video test scores (mean ± SD: 86.25 ± 17.58) were significantly higher than postpamphlet scores (72.08 ± 26.33, p < 0.0001). Older patients, in particular, showed greater improvement in comprehension after watching the video. Descriptive analysis of the feedback questionnaire on the video indicated strong agreement (average score: 4.240 ± 0.816 on a five-point Likert scale) regarding its greater effectiveness compared with the pamphlet in aiding SDM. Patients also rated the video-assisted SDM process positively (average score: 4.521 ± 0.5443 on a five-point Likert scale), highlighting increased trust and improved communication with healthcare providers. These findings underscore the value of video-assisted SDM in patient education and decision-making processes, potentially improving treatment outcomes and patient satisfaction in dermatologic care.

Capillary malformation-arteriovenous malformation (CM-AVM) is a genetic condition predominantly attributed to variations in the RASA1 or EPHB4 genes. We identified three genetic variations: a variation in the RASA1 (c.2603+1G>A) and two novel variations in the EPHB4 (c.53-2A>G and c.2222T>C), expanding the spectrum of variants associated with CM-AVM. Additionally, we found that the presence of EPHB4 variations in these two families, alongside a documented history of Bier spots, highlights the impact of genetic factors on disease phenotype. We also conducted 595 nm pulsed dye laser therapy on the proband 2, and observed that facial telangiectasia was significantly reduced after the laser treatment. We aim to enhance the understanding of the disease through case studies of three families.

Non-IgA vasculitis is a rare disease that belongs to the group of small-vessel vasculitides. Due to nomenclature and classification changes introduced in 2018, there are few published data under this name. The aim of this study is to characterize non-IgA vasculitis as an independent vasculitis entity in terms of demographic, clinical, and treatment-related features. A retrospective data analysis of patients with biopsy-confirmed non-IgA vasculitis treated at the Department of Dermatology at the University Medical Center Hamburg-Eppendorf between January 1, 2018, and December 31, 2022, was performed. A total of 28 patients with non-IgA vasculitis were included; 53.6% (15/28) were women and 42.9% (12/28) were older than 71 years. Previous infection as a possible triggering factor was found in 42.6% (12/28) of the cases. Palpable purpura was the most common skin finding (78.6%, 22/28) and 28.6% patients (8/28) had skin lesions above the waist. On direct immunofluorescence, C3 (89.3%, 25/28) was the most frequent deposition, followed by fibrinogen (71.4%, 20/28) and IgM (53.6%, 15/28). Hospitalization was required in 85.7% (24/28), with a mean hospital stay of 9.4 ± 4.1 days. No fatal courses were reported. This study is the first characterization of non-IgA vasculitis based on patient cases from Germany and contributes to a better understanding of non-IgA vasculitis as an independent entity. Non-IgA vasculitis primarily affects older patients of both sexes, with most cases having an identifiable trigger. Our results indicate that cutaneous manifestations often extend beyond the lower legs. Treatment is usually required in the inpatient setting and requires a longer stay than other dermatological conditions. With proper treatment, the disease is not expected to be fatal.

Over a 14-year period from 2010 to 2023, we treated 86 psoriasis patients with various biological agents at Asahikawa City Hospital, and 12 malignancies occurred in 11 of the patients. The numbers of malignancies by organ were as follows: four urogenital, three hematological, two gastrointestinal, one breast, one thyroid, and one lung. In two patients without cancer-related symptoms, elevated serum Krebs von den Lungen-6 levels led to the detection of intrahepatic cholangiocarcinoma or thyroid cancer, and they did not have interstitial lung disease. Dermatologists should be aware of the increased incidence of malignancy in patients with psoriasis requiring treatment with biologics.

Pyoderma gangrenosum (PG) is a rapidly progressive disease characterized by deep ulcers, predominantly in the lower extremities. Adalimumab, a monoclonal antibody against tumor necrosis factor alpha, is the first drug approved for PG treatment in Japan, ahead of other countries. We conducted a multicenter, open-label, post-marketing observational study to evaluate the safety and effectiveness of adalimumab in Japanese patients with PG. Of 67 patients enrolled, 37 in the safety analysis set and 32 in the effectiveness analysis set were included in this interim analysis. (Nineteen patients whose case report forms were not collected and 11 whose data were not fixed by the data cut-off date were excluded from the study). In the safety analysis set, the mean age was 62.9 years and 86.5% of patients had comorbidities, including ulcerative colitis (21.6%), diabetes mellitus (18.9%), and hypertension (10.8%); subtypes of PG included ulcerative (n = 33), vegetative (n = 2), and pustular (n = 2). Mean exposure duration to adalimumab was 185.5 days. Systemic steroids were used before (70.3%) and during (56.8%) adalimumab treatment. The incidence proportion of overall adverse drug reactions was 18.9%. The incidence proportions of all infections and serious infections reported as adverse drug reactions were 13.5% and 10.8%, respectively. The proportion of patients with a Physician Global Assessment score (total lesions) of 0/1 at weeks 12, 26, and 52 was 42.9%, 36.8%, and 50.0%, respectively. This interim analysis revealed the characteristics of Japanese patients with PG treated with adalimumab in the actual clinical setting and the real-world safety and effectiveness of adalimumab. At the time of the interim analysis, adalimumab treatment was generally well tolerated, and no new safety concerns were detected. Further follow-up of this study will provide a more detailed understanding of the long-term safety and effectiveness of adalimumab in patients with PG refractory to conventional treatments.