Real-world adalimumab survival and discontinuation factors in hidradenitis suppurativa.

BACKGROUND AND OBJECTIVES: Survival analyses can provide valuable insights into effectiveness and safety as perceived by prescribers. Here, we aimed to evaluate adalimumab (ADA) survival and the interruption risk factors in a multicentre cohort of patients with hidradenitis suppurativa (HS). Moreover, we performed a subanalysis considering the periods before and after the onset of the COVID-19 pandemic. METHODS: We conducted a retrospective study including 539 adult patients with HS who received ADA from 1 May 2015 to 31 December 2022. Overall drug survival was analysed using Kaplan-Meier survival curves and compared between the subgroups via stratified log-rank test. Possible predictors for overall drug survival and reasons for discontinuation were assessed using univariate and multivariate Cox regression. RESULTS: Overall, 50.1% were females with a mean age of 43.5 ± 1 years and a mean BMI of 29.5 ± 6.7. At the start of ADA, 95.29% were biologic-naïve and 24.63% had undergone surgical treatment. During follow-up, 9.46% of patients required dose escalation, while 39.92% interrupted ADA. Concomitant therapy was used in 64.89% of cases. A subanalyses comparing pre- and post-pandemic periods revealed a tendency to initiate ADA treatment at a younger age, among patient with higher BMI and at a lower HS stage after COVID-19 pandemic. Interestingly, ADA demonstrated extended survival compared to previous studies, with a median overall drug survival of 56.2 months (95% CI 51.2 to 80.3). The primary causes for discontinuation were inefficacy (51.69%), followed by adverse effects (21.35%). Female sex, longer delay in HS diagnosis, higher baseline IHS4 score and concomitant spondyloarthritis were associated with poorer ADA survival or increased risk of discontinuation. CONCLUSIONS: ADA demonstrated prolonged survival (median 56.2 months). While addition of antibiotics did not have a positive effect on survival rate, basal IHS4 proved useful in predicting ADA survival.

Multiple rhabdomyomatous mesenchymal hamartomas in a patient with mosaic Barber-Say syndrome.

Barber-Say syndrome (BSS) is a rare congenital ectodermal dysplasia with few cases reported in the literature. We describe a 9-year-old boy with congenital generalized hypertrichosis and multiple rhabdomyomatous mesenchymal hamartomas (RMHs) on his nose and periocular region. Next-generation sequencing, performed in DNA from a blood sample, and RMH tissue, revealed a pathogenic variant in the TWIST2 gene, which was not detected in a salivary sample of the patient, nor in his parents. Therefore, we consider this variant as de novo mosaicism. To our knowledge, this is the first case of multiple RMHs associated with BSS.

Chronic urticaria in children under 15 years of age: Clinical experience beyond the clinical trials.

BACKGROUND/OBJECTIVES: The aim of the study was to describe the clinical characteristics, management, and outcome of a series of children with chronic urticaria (CU). METHODS: We retrospectively studied children aged <15 years diagnosed with CU in a tertiary teaching hospital in Palma de Mallorca, Spain, between January 2014 and March 2019. CU was defined as persistence of symptoms of wheals, with or without angioedema, for >6 weeks. RESULTS: Twenty-nine patients (17 girls, mean age 8 years) were included. Family history of atopy was found in 31% of the cases. In 41.3% of patients, episodes of CU were associated with angioedema. Physical triggers were found in 34.5% of the cases. Most episodes of CU were successfully managed with the recommended (60.7%) or double the recommended dose (17.2%) of H1-antihistamines. Quadruple the recommended dose of H1 antihistamines was used in six patients, five of whom were finally treated with off-label omalizumab. Treatment with anti-leukotrienes was needed in one patient. Associated thyroiditis was diagnosed in one patient, which was controlled with levothyroxine. CONCLUSIONS: Pediatric CU showed features similar to CU in adulthood, including a greater predominance in females and frequent association with personal or family history of atopy. Adult guidelines for the treatment of CU are currently extrapolated to the pediatric population. Specific tools for the assessment of disease activity and impairment of quality of life in pediatric CU are needed for use in prospective studies aimed to define treatment strategies for children with CU.

Folliculotropic mycosis fungoides in a pediatric patient with response to psoralen-ultraviolet A phototherapy.

Mycosis Fungoides is the most common cutaneous T-cell lymphoma however, it is rare in children. We present the case of a Folliculotropic Mycosis Fungoides in a 13-year-old boy which initially presented as a plaque on his face and was treated with local PUVA therapy. Afterwards the lesions spread so the treatment was changed to systemic PUVA with good response. Although the experience in the treatment of Mycosis Fungoides in pediatric patients is limited, PUVA therapy seems to be an effective and safe option.

Intralesional photodynamic therapy in hidradenitis suppurativa: Getting closer to the target.

BACKGROUND: The successful treatment of hidradenitis suppurativa (HS) with intralesional photodynamic therapy (IL-PDT) has been previously reported in some case series published a few years ago. We present additional data and patient outcome for this form of PDT. METHODS: This is an observational retrospective study including all patients diagnosed with HS and treated with IL-PDT in the Hospital Universitari Son Espases from February 2019 to February 2020. Under local or general anesthesia, 5-aminolevulinic acid (5ALA) in gel 2 % or solution 1 % was introduced in the lesion. After two hours, the lesion was intralesionally irradiated (1.4 W, fluence 168 J/cm2) with the tip of a 0.4-mm thick optical fiber connected to a continuous 630-nm diode laser. The main outcome variable was the result (remission, improvement or no change) for each lesion 3 months after the therapy. The result at 6 months and the side effects were secondary outcomes. A multivariate analysis was carried out in order to obtain adjusted odds ratios for no change vs improvement or remission at 3 months for significant variables. RESULTS: A total of 117 lesions (28 nodules and 89 fistulae) were treated in 42 patients. At 3 months, 26 lesions (22.2 %) had resolved, 73 (62.4 %) had improved in size or symptoms, and 18 (15.4 %) experienced no change. The most frequent side effects were skin burns (53 lesions, 45.3 %), and abscess formation (8 lesions, 6.8 %). In the multivariate analysis, significant variables for no benefit were: a higher International Hidradenitis Suppurativa (IHS4) score, active smoking and gluteal and inguinal location. CONCLUSIONS: Although our results did not present the level of remission of the previous studies, IL-PDT was useful in most of our patients. Early interventions in patients with lower inflammatory burden were the most valuable.