2-Hydroxyethyl methacrylate (2-HEMA) sensitization, a global epidemic at its peak in Spain?

BACKGROUND: A global epidemic of allergic contact dermatitis to (meth)acrylates has been described in relation to the widespread use of manicure products. OBJECTIVES: To evaluate the frequency of sensitization to 2-hydroxyethyl methacrylate (2-HEMA) among consecutively patch tested patients with eczema in Spain; the percentage of current relevance; the MOAHLFA index; and, the potential sources of exposure to (meth)acrylates. METHODS: From January 2019 to December 2022, 2-HEMA 2% pet. was prospectively patch tested in 24 REIDAC (Spanish Allergic Contact Dermatitis Registry) centres. RESULTS: Six thousand one hundred thirty-four patients were consecutively patch tested with 2-HEMA 2% pet. 265/6134 (4.3%) were positive. Positive reactions of current relevance were identified to involve 184/265 (69%). The efficiency (number of patch tests needed to detect relevant positive patch test reactions) was 34 (6134/184). The variable 'occupational' was found to be significantly associated with a higher risk for relevant positive reactions to 2-HEMA (OR: 10.9; 95% CI: 8.1-14.9). CONCLUSION: (Meth)acrylate sensitization is a prevalent health issue in Spain. 2-HEMA 2% pet. has been identified to be a highly effective (meth)acrylate allergy marker in the GEIDAC baseline series. The responsible authorities should implement policies guaranteeing accurate labelling of industrial, medical, and consumer materials while ensuring the enforcement of said labelling through appropriate legal means.

Contact dermatitis from glucose sensors in Spain: A multicentric approach.

BACKGROUND: Allergic contact dermatitis from glucose sensors may interfere with their ongoing application. OBJECTIVE: To evaluate a series of Spanish patients with contact dermatitis to glucose sensors regarding former sources of contact allergens, patch test results, and outcomes from the ongoing use of the device. METHODS: A series of patients with contact dermatitis from glucose sensors was investigated in eight dermatology departments across Spain (epidemiological features, brands, latency time to develop dermatitis, the ability to continue using the devices as well as the patch test results). RESULTS: Thirty patients were evaluated (mean age 20.9 years). A total of 66.7% were children and 66.7% female. Ninety per cent used Freestyle Libre (FSL). Eight of 26 (30.8%) reacted to isobornyl acrylate (IBOA) and two of 20 (10.0%) to N,N dimethylacrylamide (DMAA). The mean latency time to develop dermatitis was 9 months. Sixteen of 29 (55.2%) patients continued using the same sensor causing the reaction. Thirteen of 29 (44.8%) patients were unable to continue using the sensor because of severe reactions. Of these, five were positive to IBOA, one to IBOA and DMAA, one to DMAA, one to colophony, and one to isopropyl alcohol wipes. In one patient, the outcome was unknown. CONCLUSION: The frequency of sensitisation to IBOA and DMAA, was lower than in other European series, but similar to a previously published Spanish article. Legislation requiring manufacturers to provide information regarding the composition of medical devices and cooperate with the investigations into contact dermatitis is urgently needed.

Unexpected relevant role of gene mosaicism in patients with primary immunodeficiency diseases.

BACKGROUND: Postzygotic de novo mutations lead to the phenomenon of gene mosaicism. The 3 main types are called somatic, gonadal, and gonosomal mosaicism, which differ in terms of the body distribution of postzygotic mutations. Mosaicism has been reported occasionally in patients with primary immunodeficiency diseases (PIDs) since the early 1990s, but its real involvement has not been systematically addressed. OBJECTIVE: We sought to investigate the incidence of gene mosaicism in patients with PIDs. METHODS: The amplicon-based deep sequencing method was used in the 3 parts of the study that establish (1) the allele frequency of germline variants (n = 100), (2) the incidence of parental gonosomal mosaicism in families with PIDs with de novo mutations (n = 92), and (3) the incidence of mosaicism in families with PIDs with moderate-to-high suspicion of gene mosaicism (n = 36). Additional investigations evaluated body distribution of postzygotic mutations, their stability over time, and their characteristics. RESULTS: The range of allele frequency (44.1% to 55.6%) was established for germline variants. Those with minor allele frequencies of less than 44.1% were assumed to be postzygotic. Mosaicism was detected in 30 (23.4%) of 128 families with PIDs, with a variable minor allele frequency (0.8% to 40.5%). Parental gonosomal mosaicism was detected in 6 (6.5%) of 92 families with de novo mutations, and a high incidence of mosaicism (63.9%) was detected among families with moderate-to-high suspicion of gene mosaicism. In most analyzed cases mosaicism was found to be both uniformly distributed and stable over time. CONCLUSION: This study represents the largest performed to date to investigate mosaicism in patients with PIDs, revealing that it affects approximately 25% of enrolled families. Our results might have serious consequences regarding treatment and genetic counseling and reinforce the use of next-generation sequencing-based methods in the routine analyses of PIDs.

Determination of volatile compounds in cider apple juices using a covalently bonded ionic liquid coating as the stationary phase in gas chromatography.

A chromatographic method for the separation of volatile compounds in Asturian cider apple juices has been developed. For this separation purpose, a monocationic imidazolium-based ionic liquid bearing a reactive terminal iodine atom was synthesized by a quaternization-anion exchange chemical sequence. Next, the gas chromatography (GC) stationary phase was prepared by covalently linking the imidazolium monolith to the reactive silanol groups of the inner capillary wall at 70 °C. This coated GC column exhibited good thermal stability (290 °C), as well as good efficiency (2000 plates/m) in the separation of volatile compounds from Asturian apple cider juices, and was characterized using the Abraham solvation parameter model. The intra-day and inter-day precision of the chromatographic method was evaluated, obtaining relative standard deviations from 3.7 to 12.9% and from 7.4 to 18.0%, respectively. Furthermore, recoveries from 82.5 to 122% were achieved. Graphical Abstract Covalent bonding of an ionic liquid to inner column wall led to a great improvement of the separation efficiencies of stationary phases in gas chromatography.

Muckle-Wells Syndrome: A Case Report with an NLRP3 T348M Mutation.

Autoinflammatory syndromes are a recently described group of conditions caused by mutations in multiple genes that code for proteins of the innate immune system. Cryopyrin-associated periodic syndromes are autoinflammatory diseases comprising three clinically overlapping disorders: familial cold urticaria syndrome, Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease. MWS is characterized by a moderate phenotype with fever, rash, arthralgia, conjunctivitis, sensorineural deafness, and potentially life-threatening amyloidosis. We report a 5-year-old girl with MWS that manifested as a recurrent skin rash without fever episodes or intracranial hypertension with papilledema. Genetic analysis revealed a T348M mutation of the NLRPR 3 gene in the patient and her mother. She was successfully treated with the interleukin-1ß antagonist receptor anakinra.

[Hematologic and molecular response with dasatinib as second-line treatment in chronic myeloid leukemia (CML) with treatment failure]. / Respuesta hematológica y molecular en leucemia mieloide crónica (LMC) con falla a tratamiento con dasatinib como fármaco de segunda línea.

BACKGROUND: Chronic myeloid leukemia is a myeloproliferative disease characterized by the Philadelphia chromosome and with this, the chimeric protein BCR-ABL. The first-line treatment is imatinib, a tyrosine kinase inhibitor, that has showed good results, but with a significant percentage of treatment failure. This failure has led to second-generation tyrosine kinase inhibitors as second-line treatment such as dasatinib. OBJECTIVES: The objective of the study was to evaluate the efficacy of dasatinib as second-line treatment. MATERIAL AND METHODS: Observational, longitudinal, and retrospective study. Patients with diagnosis of chronic myeloid leukemia that presented failure to first-line treatment were included in the present study; the hematologic response was evaluated at 3, 6, and 12 months, and molecular response at 12 months of follow-up after dasatinib treatment was started. RESULTS: Of a total of 14 patients that were included in the study, a response in the white cell count of 84.6% with a mean response at 4.7 months of follow-up was observed; also 84.6% platelet response with a mean response at 4.7 months of follow-up. Molecular response was also evaluated at a 12-month follow-up, achieving a 50% response with a mean response at 11.08 months of follow-up. A survival rate of 80% at a 12-month follow-up was observed. CONCLUSIONS: The use of dasatinib as a second-line treatment is effective in achieving a sustained hematologic response of 84.6% and a molecular response in 50%, also finding a hematologic response without achieving a total molecular response.

Systemic contact dermatitis due to amethocaine following digital rectal examination.

Systemic contact dermatitis is a dermatitis that may occur in previously sensitized individuals when they are re-exposed to the allergen. Although many drugs have been implicated as a cause of systemic contact dermatitis, local anesthetics derived from caines have been rarely reported. We present a case of systemic contact dermatitis after a digital rectal examination with a urological lubricant containing amethocaine.

Dermoscopic features of melanocytic nevi in seven different anatomical locations in patients with atypical nevi syndrome.

BACKGROUND: Dermoscopic screening facilitates early detection of melanoma and is recommended in patients with multiple or atypical nevi. OBJECTIVES: To investigate whether dermoscopic features of acquired melanocytic nevi differ between six body sites (neck, axilla, pectoral area, shoulders, buttocks, legs) and the trunk. METHODS AND MATERIALS: One hundred six patients with atypical nevi syndrome from a Digital Dermoscopy Unit were evaluated for the presence of nevi in each of seven body sites, and nevi representative of each region were selected as the predominant nevi. Dermoscopic features of 684 melanocytic nevi located in seven different body sites were analyzed. RESULTS: Globular and globular-homogeneous pattern nevi showed a cephalad distribution. Nevi at the neck, axilla, shoulders, and pectoral area showed a higher proportion of globular and globular-homogeneous patterns than other locations (p < .001). Comma vessels were also more frequent on the neck, axillary area, and shoulders (p < .001). A reticular pattern was more frequent on caudal areas (legs, buttocks and lower back, and abdomen; p < .001). CONCLUSIONS: Dermoscopic differences were found in different body areas. This information should be considered in the evaluation of lesions in patients with atypical nevus syndrome.

Polymorphous low-grade adenocarcinoma in the upper lip: a well-described but infrequently recognized tumor.

Polymorphous low-grade adenocarcinoma (PLGA) is a rare malignant neoplasm arising almost exclusively in the minor salivary glands. PLGA occurs primarily in the oral cavity, especially in the palate, followed by the oral mucosa and upper lip [1,2]. Conditions involving these locations are often presented at dermatological clinics. Therefore, dermatologists should consider this entity in the differential diagnosis of the oral cavity tumors. We present a case of PLGA in the upper lip.

Proactive Effect of Algae-Based Graphene Support on the Oxygen Evolution Reaction Electrocatalytic Activity of NiFe.

The preparation of graphene materials from biomass resources is still a challenge, even more so if they are going to be employed as supports for electrocatalysts for water splitting. Herein, we describe the preparation and characterization of graphene oxides (GOs) from solid macroalgae waste obtained after processing an agar-agar residue. The structural and morphological characterization of the obtained GO confirm the presence of a lamellar material that is composed of few layers with an increased number of heteroatoms (including nitrogen) if compared with those observed in a GO obtained from graphite (reference). Three-dimensional electrodes were prepared from these GOs by depositing them onto a fibrous carbon paper, followed by electrodeposition of the catalyst, NiFe. The electrocatalytic performance of these hybrid systems for the oxygen evolution reaction (OER) showed a proactive effect of both graphene materials toward catalysis. Moreover, the electrode prepared from the algae-based graphene showed the highest electrocatalytic activity. This fact could be explained by the different structure of the algae-based graphene which, due to differences in the nucleation growth patterns and electroactive sites developed during the electrodeposition process, produced more reactive NiFe species (higher oxidation state).

Addressing the unmet needs in patients with type 2 inflammatory diseases: when quality of life can make a difference.

Background: Patients with asthma (AS), atopic dermatitis (AD), allergic rhinitis (AR), eosinophilic esophagitis (EoE), chronic rhinosinusitis with nasal polyps (CRSwNP), chronic urticaria (CU), non-steroidal anti-inflammatory drugs-exacerbated respiratory disease (N-ERD), and certain phenotypes of chronic obstructive pulmonary disease (COPD), among others, have a common underlying pathogenesis known as Type 2 inflammation (T2i). These diseases often coexist with other T2i conditions and have a substantial impact on the quality of life (QoL) of patients. However, limited data on patients' experiences, perspectives, and current management of T2i diseases have been published thus far. Aims: This survey, promoted by the patient-driven T2i Network Project, aimed at identifying the common drivers and challenges related to the QoL of patients with T2i diseases by putting the patient's perspective at the force and including it in the design of new care strategies. Methodology: An anonymous online survey was carried out through convenience sampling between May and June 2023. The survey was codesigned by members of different patient associations, healthcare professionals and healthcare quality experts, and implemented using EUSurvey and distributed through eight patient associations from Spain. The survey consisted of 29 questions related to the participant's sociodemographic features, a series of self-reported multiple choice or rating scale questions, including diagnosis, QoL measures, disease severity, healthcare resource utilization, and quality of care. Results: The survey included 404 participants, members from eight patient associations, the majority of whom had moderate-to-severe self-reported disease severity (93%) and one or more coexisting pathologies related to T2i (59%). Patients with more than one pathology had a significantly greater impact on QoL than those with only one pathology (p < .001). Participants with self-reported severe symptoms reported significantly worse QoL than those with mild-to-moderate severity (p < .001). More than half of the patients (56%) felt constantly bothered by the unpredictability of their illness caused by potential exposure to known or unknown disease triggers. The lack of coordination between specialists and primary care was also expressed as an area of dissatisfaction by participants, with 52% indicating a complete lack of coordination and 21% indicating an average coordination. Conclusion: This article reports the initial findings of a patient-led initiative, which highlights the common QoL challenges faced by individuals with type 2 inflammation-related diseases and emphasizes the importance of further clinical research to improve the management of this patient group. Considering the significant impact on QoL, a multidisciplinary approach integrated into new healthcare protocols has the potential to improve patient management and QoL, shorten the time to diagnosis and reduce healthcare resource utilization.

Long lasting interstitial generalized granuloma annulare on sun-exposed areas.

Granuloma annulare (GA) is a benign inflammatory dermatosis of unknown etiology and chronic course, with different clinical variants. The cases associated with a photodistributed pattern are rare and show a palisading histopathological pattern. We report a case of generalized GA affecting sun-exposed areas with an interstitial pattern. The patient has been followed-up for 6 years, presenting a recurrent course of the disease, with a good response to chloroquine and relapse when no treatment was prescribed.

Experimental design applied to the analysis of volatile compounds in apple juice by headspace solid-phase microextraction.

A simple and fast method based on solid-phase microextraction (SPME) followed by fast gas chromatography (Fast GC) has been developed for the analysis of volatile compounds in Asturian apple juices employed in the cider production. Three different fiber coatings have been checked (PDMS, PDMS-DVB and CAR-PDMS) and PDMS-DVB has been presented to be the most suitable one. Experimental design has been employed in the optimization of extraction factors and robustness assessment. The use of Fast GC allowed the separation of 14 compounds (esters, aldehydes and alcohols) in approximately 4 min, clearly reducing the analysis time when compared to conventional GC. Good linearity, recoveries and repeatability of the solid-phase microextraction were obtained with r(2) values, recoveries and relative standard deviations ranging from 0.9822 to 0.9998, 83.2 to 109.8% and 0.5 to 11.7%, respectively, using standard solution.

Managing urticaria in the emergency department: recommendations of a multidisciplinary expert panel. / Manejo de la urticaria en urgencias: recomendaciones de un grupo multidisciplinar de expertos.

Urticaria and angioedema account for many visits to emergency departments. It is important to diagnose and treat them properly at this level of care and to suggest treatments and guidance that can make additional visits unnecessary. A panel of experts in dermatology and emergency medicine reviewed the main guidelines and publications on urticaria and angioedema. The panel then developed and reached consensus on practical approaches and tools for managing urticaria, angioedema, and anaphylaxis in the emergency department. The resulting statement is a guide to management, with algorithms for differential diagnosis and treatment and recommendations for patient referral. Implementing these guidelines, which are supported by the Spanish Academy of Dermatology and Venereology (AEDV) and the Spanish Society of Emergency Medicine (SEMES) will facilitate optimal management of emergency department patients with urticaria and angioedema as well as improve interdepartmental relations.

La urticaria y el angioedema ocasionan consultas frecuentes en los servicios de urgencias. Por ello, es importante que sean diagnosticados y tratados correctamente en este nivel asistencial y ofrecer un tratamiento y unas pautas de derivación adecuados para evitar visitas adicionales. Un grupo de expertos en dermatología y en medicina de urgencias y emergencias ha revisado las principales guías y publicaciones, y ha desarrollado y consensuado una herramienta práctica para el abordaje de la urticaria, el angioedema y la anafilaxia en urgencias. Presentamos una guía de manejo, un algoritmo basado en el diagnóstico diferencial, un algoritmo terapéutico y unas recomendaciones de derivación de los pacientes. La implementación de esta guía, avalada por la Sociedad Española de Dermatología (AEDV) y la Sociedad Española de Medicina de Urgencias y Emergencias (SEMES), permitirá optimizar el manejo de los pacientes con urticaria y angioedema en urgencias, y mejorará la interrelación con otros servicios.

Safety and efficacy findings from the open-label, multicenter, phase 3b, expanded treatment protocol study of ruxolitinib for treatment of patients with polycythemia vera who are resistant/intolerant to hydroxyurea and for whom no alternative treatments are available.

Ruxolitinib was recently approved for the treatment of patients with polycythemia vera who are resistant/intolerant to hydroxyurea based on data from the RESPONSE studies. This phase 3b, Expanded Treatment Protocol study (NCT02292446) of ruxolitinib for hydroxyurea-resistant/intolerant patients with polycythemia vera (N = 161: median exposure = 25.1 weeks) further evaluated the safety of ruxolitinib. Adverse events (AEs) led to dose adjustment/interruption in 37.9% of patients and study drug discontinuation in 8.7% of patients. The most common hematologic AEs included anemia and thrombocytosis; while headache and diarrhea were the most frequent nonhematologic AEs. At week 24, 45.3% of patients achieved hematocrit control; hematologic remission was seen in 18% of patients. At least, 50% of reduction in spleen length was achieved in 86.7% of patients from baseline at any time. The observed safety profile of ruxolitinib was consistent and the efficacy results were similar to the observed values in the RESPONSE studies.

Extraskeletal cutaneous chondroblastic osteosarcoma: a case report.

Extraskeletal osteosarcoma is an uncommon neoplasm that usually arises in the deep soft tissues, especially in the lower extremities, with rare cases involving the subcutis or dermis. We report a 60-year-old man with an enlarging cutaneous mass in the right lower thigh. An incisional skin biopsy showed a well-defined, but non-encapsulated neoplasm, characterized by extensive cartilage with marked cellularity, atypia and high mitotic activity, involving the dermis and subcutis. Although osteoid or bone was not observed, a diagnosis of cutaneous chondroblastic osteosarcoma was suggested after excluding an origin in bone or other primary tumor sites by imaging techniques. Histopathologic features of the surgical specimen were characteristic, with a predominant high-grade chondroid component together with focal fibrosarcomatous zones, areas of multinucleated giant cells and foci of bone and osteoid formation. The patient underwent postoperative chemotherapy and is currently alive with pulmonary metastases 15 months after surgery. In summary, we report a unique case of cutaneous osteosarcoma of the chondroblastic subtype, diagnosed on incisional skin biopsy despite the absence of osteoid or bone during tumor sampling. As a rule, when an obviously malignant 'chondrosarcoma' is identified, one should always consider this entity.