The Journal of dermatology最新文献

The advent of immune checkpoint inhibitors (ICIs) and targeted therapeutic agents (TTAs) has improved melanoma treatment. With the ongoing development of novel drugs, treatment options are expected to expand further. However, this is also anticipated to increase the financial burden on patients. Changes in medical costs for melanoma patients have not yet been examined in Japan. Therefore, we herein conducted a retrospective observational study using data from a Japanese nationwide database. The dataset comprised 14 727 patients diagnosed with melanoma between April 2008 and June 2023. The mean monthly cost per patient was calculated and changes were evaluated. The results obtained showed that the mean monthly cost per patient has consistently increased, and a breakdown revealed that drug costs for ICIs and TTAs accounted for a significant percentage of these costs. Furthermore, the usage rates of ICIs and TTAs have steadily increased since their approval in 2014, with a notable rise being observed in 2018 when adjuvant therapies were approved in Japan. An interrupted time series analysis further confirmed a significant increase in medical costs around 2018. While ICIs and TTAs represent major advances in melanoma treatment, their increasing use underscores the need for a careful evaluation of both clinical benefits and economic sustainability.

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence-based medicine techniques and aimed at improvement of the outcomes. Clinical questions (CQs) were decided based on the important clinical issues. For document retrieval, key words for literature searches were set for each CQ and literature published from 1980 to the end of December 2020 was searched in PubMed, and Japana Centra Revuo Medicina (JCRM). The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution Service (Minds) technique. A total of 38 CQs were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy, and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, pathological diagnosis deciding the diagnosis, and causal genes of vascular anomalies. Thus, the Japanese Clinical Practice Guidelines for Vascular Tumors, Vascular Malformations, Lymphatic Malformations, and Lymphangiomatosis 2022 have been prepared as the evidence-based guidelines for the management of vascular anomalies.

Generalized pustular psoriasis (GPP) is a rare and potentially life-threatening autoinflammatory skin disease characterized by widespread pustules and systemic inflammation. Accurate disease severity assessment is essential for appropriate management. To evaluate GPP severity, several scoring systems have been developed, including the Generalized Pustular Psoriasis Physician Global Assessment (GPPGA), Generalized Pustular Psoriasis Area and Severity Index (GPPASI), and Investigator's Global Assessment (IGA). In Japan, the Japanese Dermatological Association (JDA) score is commonly used, incorporating systemic inflammatory parameters, such as fever, serum C-reactive protein (CRP) level, and serum albumin level with skin conditions. This study aimed to compare the JDA score with other severity assessment tools and examine their correlation with the length of hospitalization. We retrospectively analyzed 23 hospitalizations of 13 patients with GPP admitted to Nihon University Itabashi Hospital between 2014 and 2024. The JDA score showed a significant positive correlation with the length of hospitalization (r_s = 0.482, p = 0.023), whereas the GPPGA, GPPASI, and IGA scores did not show any significant correlation. CRP levels, albumin levels, and the CRP/albumin ratio were significantly correlated with the length of hospitalization and GPPGA and GPPASI scores. These findings suggest that the JDA score, which integrates cutaneous symptoms and systemic inflammatory parameters, provides a more comprehensive and clinically relevant assessment of GPP severity than other scoring systems. Additionally, serum CRP and albumin levels should be considered in the evaluation of disease activity and prediction of clinical outcomes.

Porphyrias are rare metabolic disorders caused by decreased or increased activity of one of the nine enzymes involved in the heme biosynthetic pathway, resulting in the accumulation of porphyrins or their precursors. Although nine subtypes are currently recognized, substantial overlap in clinical manifestations often makes diagnosis challenging. This article presents the English version of the first national clinical practice guidelines for porphyrias in Japan, originally published in 2025 by the Japanese Dermatological Association. The guidelines were developed by the Clinical Practice Guideline Committee, established as part of the Research Project on Intractable Diseases of the Ministry of Health, Labour and Welfare of Japan. Diagnostic and severity assessment criteria were based on those proposed by the Ministry research group. Clinical questions (CQs) were formulated, and recommendations were developed according to the principles of evidence-based medicine, incorporating both domestic and international evidence. The guidelines provide diagnostic criteria, severity classification, and evidence-based recommendations for the management of porphyrias. Although causative genes have been identified, the pathophysiology remains incompletely understood, and no curative therapy is currently available, and liver transplantation may be required in severe cases with liver failure. These guidelines aim to provide practical guidance for clinicians managing porphyrias in Japan. Future revisions will be necessary in response to advances in diagnostic technologies and therapeutic strategies.

Trichophyton rubrum is a well-known causative agent of superficial dermatophytosis that is typically confined to the stratum corneum. However, in rare cases, it can infiltrate the dermis and cause disseminated dermatophytosis, particularly in immunocompromised patients. Here, we report a case of disseminated trichophytosis with vascular invasion that led to the dissemination of the pathogen to distant skin sites. A 51-year-old Japanese woman with a history of tinea corporis three years earlier and systemic lupus erythematosus, treated with long-term methylprednisolone, developed multiple subcutaneous nodules, abscesses, and ulcers in both lower extremities. Histopathological examination revealed extensive necrosis in the dermis and subcutaneous tissue, with clusters of filamentous fungi exhibiting asymmetric branching infiltrating both within and outside the vascular walls. Culture and genetic analyses identified Trichophyton rubrum, leading to the diagnosis of disseminated trichophytosis. Invasive aspergillosis was initially suspected, and the patient was treated with voriconazole; however, the treatment proved ineffective. Ultimately, the patient succumbed to gastrointestinal hemorrhage due to malignant lymphoma. Here, we present a case report and literature review of disseminated trichophytosis.