Nationwide prospective data on the clinical management of cutaneous angiosarcoma in Japan are lacking. We analyzed 3 years of data from the Japan Skin Cancer Registry Study, a registry involving 86 institutions. Analyses were restricted to the 335 patients registered at least 12 months before the follow-up data cutoff, a criterion independent of outcome. The median age was 79 years, 66.6% were male, and 74.0% of lesions were located on the scalp. The median tumor diameter was 8.0 cm. At diagnosis, lymph node and distant metastases were present in 14.0% and 12.5%, respectively. Radiotherapy was administered to 78.8%, systemic therapy to 72.8%, and surgery to 14.6%. The most common treatment combination was radiotherapy plus systemic therapy without surgery (54.6%). Median overall survival was 29.0 months, with 1- and 2-year survival rates of 73.8% and 54.8%, respectively. Overall survival was 33.0 months without metastasis, 20.0 months with lymph node metastasis, and 10.0 months with distant metastasis. Distant metastasis (hazard ratio 2.66; 95% confidence interval 1.63-4.33), tumor diameter (1.70 per unit of log-transformed diameter; 1.30-2.22), and age (1.03 per year; 1.01-1.05) were independent prognostic factors; lymph node metastasis (1.59; 0.96-2.61) and male sex (1.46; 0.96-2.22) were not. Two-year overall survival was higher in the surgery group (81.5% vs. 49.6%), but was no longer statistically significant after 1:1 propensity score matching (79.7% vs. 61.6%; hazard ratio 0.53; 0.24-1.20), although survival remained numerically higher in the surgery group. The objective response rate to systemic therapy declined from 57.7% in the first line to 23.0% in the second line and 6.7% in the third line. In Japan, cutaneous angiosarcoma is most commonly managed with radiotherapy and taxane-based systemic therapy rather than surgery. The survival advantage in the unadjusted analysis may partly reflect imbalances in background factors and selection of patients with a favorable prognosis for surgery.

Vunakizumab is a humanized anti-IL-17A monoclonal antibody approved in China for moderate-to-severe plaque psoriasis. Real-world data on its effectiveness and predictors of response remain scarce. This prospective, single-center, observational study enrolled 34 adults receiving vunakizumab (240 mg subcutaneously at weeks 0, 2, and 4, then every 4 weeks). PASI, PGA, BSA, and DLQI were assessed at baseline and weeks 2, 4, 8, and 12. Univariate logistic regression with Benjamini-Hochberg correction screened baseline predictors of week-4 PASI 90 and week-12 PASI 100. A separate stratification analysis compared week-12 PASI 100 rates between BMI groups using the Chinese-specific threshold of 24 kg/m2 (Fisher exact test). All 34 patients completed the 12-week follow-up (79.4% male; median age 37.5 years; median baseline PASI 10.0). At week 12, PASI 75, PASI 90, and PASI 100 rates were 100%, 91.2%, and 64.7%, respectively, and PGA 0/1 was 100%. Median DLQI decreased from 6.0 at baseline to 0 at week 12 (p < 0.001). Adverse events occurred in 17.6% of patients, all Grade 1. In exploratory analysis, higher BMI was associated with lower week-4 PASI 90 rates (q = 0.032), though the association with week-12 PASI 100 rates did not survive Benjamini-Hochberg correction (q = 0.450). On BMI stratification, normal-weight patients had higher week-12 PASI 100 rates than overweight or obese patients (92.9% vs. 45.0%, p = 0.009). Vunakizumab achieved high PASI 90 and PASI 100 response rates at week 12, with adverse events limited to Grade 1. In this small exploratory cohort, higher BMI was associated with a lower probability of PASI 100 at week 12. Whether this association is reproducible and, if so, whether it should inform patient selection requires prospective evaluation in larger cohorts.

Basal cell carcinoma (BCC) and intradermal nevus (IN) can be difficult to distinguish clinically, even with dermoscopy, because IN may exhibit dermoscopic features similar to those of BCC. In contrast, the pearly appearance characteristic of BCC is usually absent in IN and may serve as an important diagnostic clue. We hypothesized that attenuation of the overlying epidermis might contribute to this appearance by enhancing surface reflection and increasing the visibility of underlying dermal components. We retrospectively evaluated 27 non-ulcerated facial BCCs (25 nodular and 2 morpheaform) and compared them with facial INs (n = 19), malignant melanomas (MM; n = 6), Merkel cell carcinomas (MCC; n = 3), and mixed tumors (MT; n = 4). Epidermal thickness and hair follicle density were quantitatively assessed in lesional and adjacent normal skin. Dermal mucin deposition and collagen staining intensity were also evaluated. The lesional epidermis was thinner than the adjacent normal epidermis in all 27 BCCs. The lesional-to-normal epidermal thickness ratio was significantly lower in BCC than in IN. The lesional-to-normal hair follicle density ratio was also significantly lower in BCC than in IN. Superficial dermal mucin deposition was observed in all BCCs but not in the other tumor types, whereas the lesional-to-normal collagen staining intensity ratio was comparable between BCC and IN. A pearly appearance was observed in all 25 nodular BCCs but not in the two morpheaform BCCs despite epidermal attenuation. None of the other tumor types exhibited a pearly appearance, including MM lesions with epidermal attenuation. These findings demonstrate that epidermal attenuation is a consistent histopathological feature of non-ulcerated nodular BCC and raise the possibility that it contributes to the characteristic pearly appearance of nodular BCC. However, because epidermal attenuation was also observed in lesions without a pearly appearance, its actual contribution remains uncertain and requires further investigation.

Psoriasis is a chronic inflammatory disease associated with multiple comorbidities, but its association with cataract remains unclear. We aimed to examine the risks of cataract and cataract surgery in psoriasis patients and the associations of ultraviolet phototherapy with these outcomes. Using the Taiwan National Health Insurance Research Database (2012-2023), we conducted a retrospective cohort study of patients with newly diagnosed psoriasis who were matched to non-psoriasis controls by age, sex, and index year. Cox proportional hazards models were used to estimate hazard ratios (HRs) for cataract and cataract surgery. A nested case-control analysis was then performed among psoriasis patients who developed cataract or underwent cataract surgery, with matching to psoriasis patients without these outcomes. Conditional logistic regression was used to evaluate the associations of psoriasis-related treatments, including phototherapy and systemic treatments, with cataract and cataract surgery. A total of 61 622 psoriasis patients and 61 622 matched controls were included. Psoriasis was associated with increased risks of cataract (adjusted HR, 1.32; 95% confidence interval [CI], 1.24-1.40) and cataract surgery (adjusted HR, 1.41; 95% CI, 1.30-1.53). These associations were generally consistent across subgroups stratified by age, sex, and psoriatic arthritis status. In the nested case-control analysis, both psoralen plus ultraviolet A (PUVA) (adjusted OR, 1.88) and ultraviolet B (UVB) phototherapy (adjusted OR, 1.22) were associated with increased odds of cataract, without a clear dose-response relationship. In contrast, systemic treatments, including biologics/Janus kinase inhibitors (adjusted OR, 0.49) and conventional disease-modifying antirheumatic drugs (adjusted OR, 0.74) were associated with reduced odds of cataract. Similar findings were observed for cataract surgery. Psoriasis was associated with increased risks of cataract and cataract surgery. PUVA and UVB phototherapy were associated with higher odds of cataract and cataract surgery, whereas systemic treatments were associated with lower odds.

Pemphigus is a rare autoimmune blistering disease for which rituximab is a first-line treatment, yet most safety data derive from lymphoma and rheumatoid arthritis cohorts. This study assessed hypogammaglobulinemia and infection during rituximab treatment, the baseline predictors of new-onset hypogammaglobulinemia, and whether desmoglein antibody titers and total immunoglobulin levels fall together. In this single-center retrospective cohort study, the records of 97 patients given rituximab for pemphigus between January 2015 and June 2026 were reviewed. Patients who received intravenous immunoglobulin were excluded, leaving 69 patients for outcome analyses. Among 51 patients with a normal baseline immunoglobulin G level, new-onset hypogammaglobulinemia, defined as a fall below 700 mg/dL after treatment, occurred in 5 (10%), and no patient reached the severe threshold of 400 mg/dL. Seven further patients were already below 700 mg/dL before treatment. Patients who developed new-onset hypogammaglobulinemia were older and had lower baseline immunoglobulin G and immunoglobulin M levels (areas under the curve 0.85, 0.83, and 0.81). Patients with hypogammaglobulinemia had received more corticosteroid per cycle, so a contribution of corticosteroids cannot be excluded. Late-onset neutropenia was not detected (0 of 69). Infection occurred in 5 of 69 patients (7%) and severe infection in 2 of 69 (3%). 6 months after induction, desmoglein 1 and desmoglein 3 antibody titers had fallen to 16% and 19% of baseline (p < 0.001), whereas immunoglobulin G remained at 97% (p = 0.46), independently of hypogammaglobulinemia. Hypogammaglobulinemia remained mild and the serologic response was not mirrored in total immunoglobulin levels. Advanced age and low baseline immunoglobulin levels are candidate markers of risk. These findings come from a retrospective single-center cohort and require confirmation in larger series.

To investigate whether 10-year changes in nailfold videocapillaroscopy (NVC) findings are associated with changes in clinical characteristics in systemic sclerosis (SSc). This retrospective single-center study included 84 patients with SSc who underwent NVC assessment at baseline and after 10 years. NVC patterns were classified as normal, non-specific, early, active, or late. Patients were categorized by longitudinal NVC changes. Associations of NVC-change groups with changes in clinical characteristics including modified Rodnan skin score (mRSS), digital ulcers, interstitial lung disease, percent predicted vital capacity (%VC), and percent predicted diffusing capacity for carbon monoxide (%DLco) were examined. Over 10 years, NVC findings improved in 19 patients, remained unchanged in 41, and worsened in 24. Change in mRSS differed significantly among the groups, with greater improvement in the improved group than in the unchanged group (p = 0.0140) and the worsened group (p = 0.0161). Changes in digital ulcers were not associated with NVC-change groups. Among 47 patients with longitudinal %DLco data, Δ%DLco differed significantly among the groups, with the greatest decline in the worsened group (p = 0.0067), whereas Δ%VC did not differ significantly. NVC-change group was significantly associated with mRSS at 10 years after adjustment for baseline mRSS and disease duration (p = 0.0291), but not with Δ%DLco after adjustment for interstitial lung disease and pulmonary hypertension at 10 years (p = 0.0684). In conclusion, long-term NVC changes may occur in both directions may reflect the long-term course of skin sclerosis.

CTSC-syndromic epidermal differentiation disorder is a rare autosomal recessive genodermatosis caused by loss-of-function mutations in the cathepsin C gene. It is characterized by palmoplantar keratoderma and early-onset periodontal destruction, and no standardized disease-specific therapy has been established. We report a 3-year-old boy with disease onset in infancy, presenting with palmoplantar and generalized erythematous scaly lesions, severe pruritus, sleep disturbance, and nail roughness. Whole-exome sequencing followed by Sanger sequencing identified compound heterozygous variants in the cathepsin C gene: c.774C>G inherited from his father and c.328G>T inherited from his mother. Treatment with cyclosporine combined with systemic retinoids failed to prevent disease progression. After pretreatment evaluation and written informed consent from his guardian, secukinumab was administered subcutaneously at a dose of 75 mg at weeks 0, 1, 2, 3, and 4, followed by once every 4 weeks thereafter. After 12 weeks of treatment, the patient showed marked improvement in clinical manifestations, quality-of-life scores, and disease severity scores, with no infectious complications during treatment at our institution. After secukinumab was continued locally, an infection involving the right foot and ankle led to treatment discontinuation, and a pulmonary infection was reported during later follow-up.

IgA vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is a small-vessel vasculitis characterized by IgA-dominant immune deposits in the skin, kidney, gastrointestinal tract, and joints, often following infections, particularly β-hemolytic streptococci. Nephritis-associated plasmin receptor (NAPlr), a streptococcal glyceraldehyde-3-phosphate dehydrogenase, has been identified as a nephritogenic protein in post-streptococcal acute glomerulonephritis, binding plasmin and maintaining its proteolytic activity, thereby promoting tissue injury. However, the role of NAPlr in IgAV pathophysiology remains unclear. In this study, we investigated histological staining for NAPlr and plasmin activity in IgAV cutaneous lesions and evaluated circulating anti-NAPlr antibodies. Skin specimens from patients with IgAV were analyzed by immunofluorescence and in situ zymography, and serum anti-NAPlr antibodies (IgG, IgM, and IgA) were measured by enzyme-linked immunosorbent assay in 21 patients with IgAV and 18 healthy donors. Positive staining for NAPlr and plasmin activity was observed in dermal lesions in 7 of 11 patients with IgAV, but was minimal in control skin. Patients with IgAV showed significantly elevated serum levels of anti-NAPlr IgG and IgA antibodies compared with healthy donors. Anti-NAPlr IgG levels positively correlated with serum creatinine and negatively correlated with CH50, with a nonsignificant inverse association with estimated glomerular filtration rate (eGFR). Serum galactose-deficient IgA1 levels were also significantly increased in patients with IgAV, and were positively correlated with anti-NAPlr IgA levels but negatively correlated with C4. These findings demonstrate the presence of a protein that is stained by anti-NAPlr antibody in IgAV cutaneous lesions alongside elevated circulating anti-NAPlr antibodies, suggesting that immune responses to NAPlr may contribute to IgA-mediated vascular inflammation, potentially through complement activation.