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.

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.

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

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. (AU)

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. (AU)

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.

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.

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.

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.

Pustulosis cutánea diseminada / Generalized cutaneous pustulosis

No disponible

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.

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.