Syringotropic mycosis fungoides.

Brief description of a syringotropic mycosis fungoides.

Omalizumab in the treatment of bullous pemphigoid resistant to first-line therapy: a French national multicentre retrospective study of 100 patients.

BACKGROUND: Interest in the use of omalizumab to treat bullous pemphigoid (BP) in the event of resistance or contraindication to conventional therapies is currently based on limited evidence. OBJECTIVES: To assess the effectiveness and safety of omalizumab in BP and to identify predictive factors in response to treatment. METHODS: We conducted a French national multicentre retrospective study including patients with a confirmed diagnosis of BP treated with omalizumab after failure of one or several treatment lines. We excluded patients with clinically atypical BP, as per Vaillant's criteria. The criteria for clinical response to omalizumab were defined according to the 2012 international consensus conference. Anti-BP180-NC16A IgE enzyme-linked immunosorbent assay was performed on sera collected before initiating omalizumab, when available. RESULTS: Between 2014 and 2021, 100 patients treated in 18 expert departments were included. Median age at diagnosis was 77 years (range 20-98). Complete remission (CR) was achieved in 77% of patients, and partial remission in an additional 9%. CR was maintained 'off therapy' in 11.7%, 'on minimal therapy' in 57.1%, and 'on non-minimal therapy' in 31.2%. Median time to CR was 3 months (range 2.2-24.5). Relapse rate was 14%, with a median follow-up time of 12 months (range 6-73). Adverse events occurred in four patients. CR was more frequently observed in patients with an increased serum baseline level of anti-BP180-NC16A IgE (75% vs. 41%; P = 0.011). Conversely, urticarial lesions, blood total IgE concentration or eosinophil count were not predictive of CR. Patients with an omalizumab dosage > 300 mg every 4 weeks showed a similar final outcome to those with a dosage ≤ 300 mg every 4 weeks, but control of disease activity [median 10 days (range 5-30) vs. 15 days (range 10-60); P < 0.001] and CR [median 2.4 months (range 2.2-8.2) vs. 3.9 months (range 2.3-24.5); P < 0.001] were achieved significantly faster. CONCLUSIONS: We report the largest series to date of BP treated by omalizumab and confirm its effectiveness and safety in this indication. Serum baseline level of anti-BP180-NC16A IgE may predict response to treatment.

Cemiplimab for Locally Advanced and Metastatic Cutaneous Squamous-Cell Carcinomas: Real-Life Experience from the French CAREPI Study Group.

Although cemiplimab has been approved for locally advanced (la) and metastatic (m) cutaneous squamous-cell carcinomas (CSCCs), its real-life value has not yet been demonstrated. An early-access program enrolled patients with la/mCSCCs to receive cemiplimab. Endpoints were best overall response rate (BOR), progression-free survival (PFS), overall survival (OS), duration of response (DOR) and safety. The 245 patients (mean age 77 years, 73% male, 49% prior systemic treatment, 24% immunocompromised, 27% Eastern Cooperative Oncology Group performance status (PS) ≥ 2) had laCSCCs (35%) or mCSCCs (65%). For the 240 recipients of ≥1 infusion(s), the BOR was 50.4% (complete, 21%; partial, 29%). With median follow-up at 12.6 months, median PFS was 7.9 months, and median OS and DOR were not reached. One-year OS was 73% versus 36%, respectively, for patients with PS < 2 versus ≥ 2. Multivariate analysis retained PS ≥ 2 as being associated during the first 6 months with PFS and OS. Head-and-neck location was associated with longer PFS. Immune status had no impact. Severe treatment-related adverse events occurred in 9% of the patients, including one death from toxic epidermal necrolysis. Cemiplimab real-life safety and efficacy support its use for la/mCSCCs. Patients with PS ≥ 2 benefited less from cemiplimab, but it might represent an option for immunocompromised patients.

Bidimensional Multiscale Fuzzy Entropy and Its Application to Pseudoxanthoma Elasticum.

OBJECTIVE: We propose a new bidimensional entropy measure and its multiscale form and evaluate their behavior using various synthetic and real images. The bidimensional multiscale measure finds application in helping clinicians for pseudoxanthoma elasticum (PXE) detection in dermoscopic images. METHOD: We developed bidimensional fuzzy entropy ( FuzEn2D) and its multiscale extension ( MSF2D) and then evaluated them on a set of synthetic images and texture datasets. Afterwards, we applied MSF2D to dermoscopic PXE images and compared the results to those obtained by bidimensional multiscale sample entropy ( MSE2D). RESULTS: The results for the synthetic images illustrate that FuzEn2D has the ability to quantify images irregularity. Moreover, FuzEn2D, compared with bidimensional sample entropy ( SampEn2D), leads to more stable results. The tests with the multiscale version show that MSF2D is a proper image complexity measure. When applied to the dermoscopic PXE images, the paired t-test illustrates a significant statistical difference between MSF2D of neck images with papules and normal skin images at a couple of scale factors. CONCLUSION: The results for the synthetic data illustrate that FuzEn2D is an image irregularity measure that overcomes SampEn2D in terms of reliability, especially for small-sized images, and stability of results. The results for the PXE dermoscopic images demonstrate the ability of MSF2D to recognize dermoscopic images of normal zones from PXE papules zones with a large effect size. SIGNIFICANCE: This work introduces new image irregularity and complexity measures and shows the potential for MSF2D to serve as a possible tool helping medical doctors in PXE diagnosis.

Duration of Efficacy Increases with the Repetition of Botulinum Toxin A Injections in Primary Axillary Hyperhidrosis: A 15-year Study in 117 Patients.

Changes in the duration of efficacy of botulinum toxin A injections for primary axillary hyperhidrosis have not been studied in depth. The aim of this study was to assess such changes. In a retrospective cohort of 220 patients, seen over a 17-year period, duration of efficacy was recorded, including duration of efficacy of first and last injections. Of 220 patients, 117 fulfilled the study criteria (79 females and 38 males, age 17-79 years, mean age 38.3 years). Patients received 3-24 injections (mean 5.5). The duration of efficacy of the first injections was 3 weeks to 30 months (median 6 months), and the duration of efficacy of the final injections was 3 weeks to 66 months (median 8 months) (p < 0.001). An increase in duration of efficacy occurred in 62% of patients with repeated injections. Of the 20 patients with a follow up ≥10 years, the mean number of treatments was 8.25/patient; in 18 of these patients the duration of efficacy increased by > 50%. In conclusion, repeated botulinum toxin A injections lead to an increase in duration of efficacy without secondary decrease.