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Background Chronic Urticaria (CU) is a debilitating disease whose treatment is mainly symptomatic. UCREX study aimed to identify CU patients’ profile, disease management and quality-of-life (QoL) in daily clinical practice in Spain. Methods Observational, 12-months prospective, multicenter study, included de novo or established CU patients attending to dermatology/allergy consultations in 39 Spanish hospitals. Main variables: Urticaria Activity Score (UAS), UAS over 7 days (UAS7). Secondary variables: CU-QoL Questionnaire (CU-Q2oL), EuroQol-5 dimensions (EQ-5D), Medical Outcomes Study Sleep (MOS-Sleep) scale, Hospital Anxiety and Depression Scale (HADS). Results 361 patients included. Of them, 176 (48.8%) considered for the main objective analysis. Mean age (SD) of 46.6 (14.2) years and 71.8% women. The year prior to inclusion, most patients (57.1%) were treated with non-sedating H1-antihistamines (NS-H1AH). At baseline, mean (SD) 3.6 (6.8) visits were registered to primary care. Mean (SD) UAS7 at baseline was 14.3 (11.0) and CU-Q2oL 24.1 (17.0) which tended to improve by 8.6 (9.7) and 13.9 (15.0), respectively, at 12-months. MOS-Sleep and EQ-5D remained steady during the study, except pain/discomfort and anxiety/depression which went from 58.7% and 49.6% to 29.6% and 26.9%, respectively. At baseline, HADS showed a mean (SD) anxiety of 8.7 (4.5) and depression 5.1 (4.4), decreasing to 7.0 (4.3) and 4.7 (4.3), respectively, at 12-months. Conclusions Although most CU patients are treated with NS-H1AH, disease activity is still important, negatively affecting patients’ QoL, work activity and healthcare resources use. An appropriate disease management could be the basis for symptoms control, QoL improvement and resources optimization.

Resumen: Antecedentes y objetivo: La actualización de las series estándar de pruebas epicutáneas debe basarse en datos objetivos de frecuencia de sensibilización de los alérgenos que componen cada batería. La última renovación de la batería estándar española se realizó en 2016 y de la europea en 2019, quedando pendiente valorar la inclusión de alérgenos emergentes. Material y método: Desarrollamos un estudio observacional, prospectivo y multicéntrico de los pacientes consecutivos del registro del Grupo Español de Investigación en Dermatitis y Alergia Cutánea sometidos a pruebas epicutáneas con hidroperóxido de linalool, hidroperóxido de limoneno, 2-hidroxi-etil-metacrilato, benzisotiazolinona, octilisotiazolinona, mezcla textil, metabisulfito sódico, propóleo, bronopol, mezcla de compuestas II, diazolidinil urea, imidazolidinil urea, decil glucósido y lauril glucósido, durante los años 2019 y 2020. Resultados: Se analizó una muestra de 4.654 pacientes estudiados con diazolidinil urea, imidazolidinil urea y bronopol, y de 1.890 pacientes con el resto de los alérgenos. El índice MOAHLFA fue: M 30%, O 18%, A 15%, H 29%, L 6,5%, F 23%, A 68%. Siete alérgenos mostraron una frecuencia de sensibilización mayor del 1%: hidroperóxido de linalool, 2-hidroxi-etil-metacrilato, benzisotiazolinona, hidroperóxido de limoneno, mezcla textil, metabisulfito sódico y propóleo. Tres alérgenos mostraron una frecuencia de relevancia presente superior al 1%: hidroperóxido de linalool, 2-hidroxi-etil-metacrilato e hidroperóxido de limoneno; para benzisotiazolinona y mezcla textil, esta frecuencia fue de entre el 0,9 y el 1%. Conclusiones: Nuestros resultados indican que debería valorarse la inclusión de siete nuevos alérgenos en la batería estándar española. Estos resultados podrían contribuir a la próxima actualización de la batería basal europea. Abstract: Background: Standard patch test series must be updated using objective data on allergen sensitization. The Spanish standard series was last updated in 2016 and the European series in 2019, and the inclusion of several emerging allergens needs to be evaluated. Material and methods: We conducted a prospective, observational, multicenter study of consecutive patients from the registry of the Spanish Contact Dermatitis and Skin Allergy Research Group (GEIDAC) who were patch tested in 2019 and 2020 with linalool hydroperoxide, limonene hydroperoxide, 2-hydroxyethyl-methacrylate, benzisothiazolinone, octylisothiazolinone, textile dye mix (TDM), sodium metabisulfite, propolis, bronopol, Compositae mix II, diazolidinyl urea, imidazolidinyl urea, decyl glucoside, and lauryl glucoside. Results: We analyzed data for 4654 patients tested with diazolidinyl urea, imidazolidinyl urea, and bronopol, and 1890 tested with the other allergens. The values for the MOAHLFA index components were 30% for male, 18% for occupational dermatitis, 15% for atopic dermatitis, 29% for hand, 6.5% for leg, 23% for face, and 68% for age > 40 years. Sensitization rates above 1% were observed for 7 allergens: linalool hydroperoxide, 2-hydroxyethyl-methacrylate, benzisothiazolinone, limonene hydroperoxide, TDM, sodium metabisulfite, and propolis. Three allergens had a current relevance rate of over 1%: linalool hydroperoxide, 2-hydroxyethyl-methacrylat, and limonene hydroperoxide. Benzisothiazolinone and TDM had a relevance rate of between 0.9% and 1%. Conclusions: Our results indicate that 7 new allergens should be considered when extending the Spanish standard patch test series. The data from our series could be helpful for guiding the next extension of the European baseline series.

Background: Standard patch test series must be updated using objective data on allergen sensitization. The Spanish standard series was last updated in 2016 and the European series in 2019, and the inclusion of several emerging allergens needs to be evaluated. Material and methods: We conducted a prospective, observational, multicenter study of consecutive patients from the registry of the Spanish Contact Dermatitis and Skin Allergy Research Group (GEIDAC) who were patch tested in 2019 and 2020 with linalool hydroperoxide, limonene hydroperoxide, 2-hydroxyethyl-methacrylate, benzisothiazotinone, octylisothiazolinone, textile dye mix (TDM), sodium metabisulfite, propolis, bronopol, Compositae mix II, diazolidinyl urea, imidazolidinyl urea, decyl glucoside, and lauryl glucoside. Results: We analyzed data for 4654 patients tested with diazolidinyl urea, imidazolidinyl urea, and bronopol, and 1890 tested with the other allergens. The values for the MOAHLFA index components were 30% for mate, 18% for occupational dermatitis, 15% for atopic dermatitis, 29% for hand, 6.5% for leg, 23% for face, and 68% for age > 40 years. Sensitization rates above 1% were observed for 7 allergens: linalool hydroperoxide, 2-hydroxyethyl-methacrylate, benzisothiazolinone, limonene hydroperoxide, TDM, sodium metabisulfite, and propolis. Three allergens had a current relevance rate of over 1%: linalool hydroperoxide, 2-hydroxyethyl-methacrylat, and limonene hydroperoxide. Benzisothiazotinone and TDM had a relevance rate of between 0.9% and 1%. Conclusions: Our results indicate that 7 new allergens should be considered when extending the Spanish standard patch test series. The data from our series could be helpful for guiding the next extension of the European baseline series. (C) 2021 AEDV. Published by Elsevier Espana, S.L.U.

Resumen Introduccion y objetivos Conocer, desde la perspectiva del paciente, los desencadenantes de la dermatitis atopica (DA), el grado de control percibido y el cumplimiento de las indicaciones medicas y su calidad de vida relacionada con la salud (CVRS). Pacientes y metodos Estudio epidemiologico, transversal, multicentrico incluyendo pacientes adultos (> 16 anos; n = 125) y pediatricos (entre 2-15 anos; n = 116) con DA de intensidad moderada-grave, mas de 12 meses de evolucion y con episodios de lesiones activas moderados-graves (escala de evaluacion global del investigador [IGA] > 2). Se analizaron los desencadenantes informados por los pacientes, el cumplimiento de las recomendaciones y el tratamiento farmacologico (TF), las diferencias en CVRS y el control percibido (U de Mann-Whitney) segun la gravedad de la DA (indice SCORAD-SCORing Atopic Dermatitis). Resultados Los desencadenantes mas frecuentes fueron: cosmeticos, ropa, acaros, detergentes/jabones y cambios de temperatura. El 47,2% de los pacientes adultos y el 39,7% de los pediatricos no aplicaban el TF desde el inicio del episodio. El TF, la hidratacion y los consejos medicos de cuidado fueron las recomendaciones mas seguidas. El 41,6 y el 27,6% (adultos y pediatricos, respectivamente) consideraba que su grado de control era insuficiente y se asocio con la gravedad de la DA en adultos (p = 0,014). Conclusiones El grado de control actual de la DA es mejorable, especialmente en adultos. Aunque los pacientes indican seguir las recomendaciones medicas, un porcentaje significativo no aplica correctamente los tratamientos. Parece necesario potenciar la educacion sobre la enfermedad y su manejo para mejorar el grado de control y potenciar su CVRS.

Introduction and objectives: To analyze the triggers of atopic dermatitis (AD), adherence to medical recommendations, disease control, and health-related quality of life (HRQOL) from the patient’s perspective. Patients and methods: This was a multicenter, cross-sectional, epidemiological study with the participation of adults (age > 16 years; n = 125) and children (age, 2-15 years, n = 116). Patients had a history of at least 12 months of moderate to severe AD with a moderate to severe flare (Investigator Global Assessment score > 2) at the time of recruitment. The Mann-Whitney U test was used to evaluate relationships between disease severity, determined according to the Scoring in Atopic Dermatitis index, and triggers reported by patients, adherence to recommendations and pharmacological therapy, HRQOL, and patient-perceived control. Results: The most common triggers were cosmetic products, clothing, mites, detergents/soaps, and changes in temperature. In 47.2% of adults and 39.7% of children, pharmacological therapy was not initiated at flare onset. Adherence was highest to pharmacological therapy, skin

Resumen Introduccion Mientras que la estandarizacion de la exploracion con pruebas de fotoalergia o fotoparche (FTP) sigue su curso en Europa, hemos realizado un estudio epidemiologico sobre la situacion actual de la fotoalergia en nuestro pais. Material y metodos Hemos recogido los resultados del FTP en 7 centros hospitalarios de Madrid, Cataluna, Galicia y Comunidad Valenciana, durante los anos 2004 y 2005. La exploracion ha incluido, al menos, la bateria estandar del Grupo Espanol de Fotobiologia (GEF), con 16 (foto)alergenos, que se han irradiado con 10 julios/ cm 2 . Hemos valorado el numero total de pacientes explorados, su sexo, la relevancia presente, pasada o desconocida de los fotoparches positivos, las reacciones cruzadas y los alergenos responsables de la fotosensibilizacion. Resultados El 39,3 % de los 224 pacientes explorados mediante FTP presentaron uno o mas fotoparches positivos. Ciento tres (71 %) se consideraron relevantes respecto a la historia clinica, 14 (9,6 %) fueron reacciones cruzadas y 28 (19,3 %) se consideraron de relevancia desconocida. Los alergenos mas prevalentes fueron los antiinflamatorios no esteroideos, particularmente el ketoprofeno (43 pacientes), seguido a distancia de la bencidamina (7 pacientes) y el etofenamato (5 pacientes). La mezcla de 4 filtros solares de la bateria estandar del GEF solo detecto 10/16 pacientes con fotoalergia a filtros solares. Los fotoparches de relevancia desconocida fueron sobre todo por antisepticos (fenticlor) y antihistaminicos topicos. Conclusiones Proponemos modificar la bateria estandar de fotoalergenos del GEF, que deberia contemplar la mayoria de los antiinflamatorios no esteroideos y filtros solares que hay en el mercado espanol. El ketoprofeno sigue siendo el fotoalergeno mas frecuente en nuestro pais. Su importancia deriva tambien de las sensibilizaciones cruzadas que puede presentar. Los filtros solares deben explorarse por separado, y no en forma de una mezcla.

Nearly fifty years after their introduction, corticoids continue to be one of the main pillars of dermatological therapy in general and in atopic dermatitis in particular. However, their use is limited by local and systemic adverse effects.There is enormous demand for anti-inflammatory agents not belonging to the corticoid group that would be useful in the different inflammatory dermatoses.In the last few years the therapeutic arsenal for atopic dermatitis has increased with two distinct groups of drugs: topical immunomodulators and leukotriene inhibitors. Both groups of drugs are new and new compounds belonging to these types of drugs will probably appear in the next few years.Among the alternatives to corticoids are the immunomodulators and the most promising of these are macrolide antibiotics. Tacrolimus (FK 506) and pimecrolimus (ASM 981) belong to this group of substances with a high capacity to inhibit T lymphocyte activation. Although they also act on other cells playing a role in atopic dermatitis (mastocytes, Langerhans' cells, B lymphocytes) their action on T lymphocytes seems to be the most important. In T lymphocytes, these drugs act by inhibiting the action of calmodulin, a vital enzyme in the activation chain of these cells that ends in the production of interleukin 2 and other proinflammatory cytokines.The accumulated evidence of the various publications seems to indicate that tacrolimus is a safe and effective treatment of atopic dermatitis in patients aged 2 years or more. The drug was approved for clinical use 3 years ago in Japan and more than 1 year ago in the United States. The number of randomized clinical trials comparing this drug with placebo or various corticoids demonstrate that its action is overwhelming. Its anti-inflammatory action is similar to that of high-potency topical corticoids. Two doses daily are required and the drug has been tested in patients with moderate-to-severe atopic dermatitis. Published data from more than 10,000 patients guarantee its safety. The most frequent short-term adverse effect is a burning sensation in the site of application lasting a few minutes.Pimecrolimus has not yet been commercialized in any country and is currently undergoing phase III clinical investigations. It has been tested in three clinical trials in patients aged more than 3 months old with mild-moderate atopic dermatitis. Its safety profile is good and similar to that of tacrolimus.The number and quality of the data provided by studies of treatment with leukotriene inhibitors (zafirlukast, montelukast and zileuton) is much lower but these substances seem to warrant further investigation. Only five small series undergoing treatment with these products have been published.

Bajo el término de displasia ectodérmica se agrupa una gran variedad de cuadros clínicos que comparten unos rasgos comunes como la afectación de uno o varios com-ponentes derivados del ectodermo y su origen congénito. En la actualidad se han descrito 154 tipos diferentes que se han distribuido en 11 grupos distintos (clasificación de Freire Maia, 1994). De todos ellos, el cuadro más frecuente es la displasia ectodérmica hipo/anhidrótica (síndrome de Christ-Siemens-Touraine). Se trata de un trastorno genético, cuya característica más sobresaliente es la ausencia, o más frecuentemente la disminución, de las glándulas sudoríparas, lo que ocasiona un aumento de la temperatura corporal, junto con otras alteraciones de la epidermis y sus anejos (pelo y uñas). Se presenta un nuevo caso de displasia ectodérmica hi-pohidrótica en un varón prematuro de 18 meses de edad que fue remitido al servicio de dermatología por presentar desde su nacimiento una piel llamativamente seca con brotes repetidos de lesiones eccematosas y liquenificadas que se resolvían con tratamiento con corticoides tópicos. En su aspecto externo resaltaba una alopecia difusa en cuero cabelludo con un pelo rubio, ralo y fino además de la ausencia de los alveolos dentarios. Junto con todo esto, la madre había apreciado una escasa sudoración y episodios de fiebre sin sintomatología acompañante preferentemente en la época estival. Con todo lo anterior creemos importante considerar el diagnóstico de displasia ectodérmica hipohidrótica ante un paciente que presente episodios de fiebre de origen desconocido. : The term ectodermal dysplasias includes many disorders that share some clinical features such as involvement of one or several ectodermal structures and congenital origin. Currently, 154 different types divided into 11 clinical subgroups (Freire Maia classification 1994) have been de-scribed. The most frequent entity is hypo- or anhidrotic ectodermal dysplasia (Christ-Siemens-Touraine syn-drome). This is a rare hereditary disease whose main characteristic is the absence, or more often the reduction, of sweat glands, leading to an increase in body temperature together with anomalies of the epidermis and its appendages (hair and nails). We present a case of hypo-hidrotic ectodermal dysplasia in a premature 18-month-old boy who was referred to our department because of markedly dry skin since birth and recurrent eczematous and lichenification lesions that had been successfully treated with topical corticosteroids. Physical examination revealed mild alopecia with sparse and fine blonde hair and the absence of dental alveoli. The boy's mother had noticed slight sweating and episodes of fever without clinical symptoms, which were more frequent in summer. Hy-pohidrotic ectodermal dysplasia should be included in the differential diagnosis of fever of unknown origin.

Resumen La dermatomiositis (DM) es una dermatomiopatia con un amplio espectro de manifestaciones, definido por Bohan et al como un conjunto objetivo de criterios clinico-analiticos. La DM se ha relacionado con neoplasias extracutaneas, especialmente con carcinoma ovarico. El termino queratodermia punctata incluye un grupo de enfermedades con diferentes etiologias, caracterizadas por lesiones hiperqueratosicas de pequeno tamano, habitualmente salpicadas en palmas y plantas. La queratodermia poroqueratosica punctata (QPP) es un tipo de queratodermia punctata definida principalmente por sus peculiares caracteristicas histopatologicas, incluyendo la presencia de lamela cornoide, similar a la observada en la poroqueratosis. La QPP ha sido considerada una enfermedad esporadica; sin embargo se ha publicado algun caso raro paraneoplasico. Presentamos por vez primera un caso de DM con datos tipicos de QPP, ambas como manifestaciones paraneoplasicas de un carcinoma ovarico. Su naturaleza paraneoplasica es claramente demostrada por la mejoria clinica observada tras la extirpacion de la neoplasia.

Bachata Even as I first approached her, I already knew That she was composed of the very substance that lies at the heart of a thousand stars; Terpsichore, the muse of dance, and Erato, the muse of love poetry, rarely visit the same people, but both are divinities that hold enormous fascination for the scientist, a rationalist little blessed with the emotional intelligence for, or even interest in, the so-called world of arts and letters. When such a person runs into them in the unpredictable labyrinths of fate, they feel deeply affected in a manner similar to how Saint Paul must have felt when he fell off his horse on the road to Damascus. Nothing is the same thereafter... Placing the powers of the word, music, and movement at the service of Eros is, on occasion, an irresistible temptation.

Dermatomyositis (DM) is a dermatomyopathy with a broad spectrum of features, defined by Bohan et al as an objective set of clinical and laboratory criteria. Dermatomyositis has been associated with extra- cutaneous neoplasias, especially with ovarian carcinoma. The term "punctate keratoderma" comprises a group of diseases with different etiologies characterized by small hyperkeratotic lesions usually scattered in palms and soles. Punctate porokeratotic keratoderma (PPK) is a type of punctate keratoderma mainly defined by its peculiar histological features, including the presence of cornoid lamella similar to the one observed in porokeratosis. Punctate porokeratotic keratoderma has been considered a sporadic disease although some rare paraneoplastic cases have been published. We report for the first time a case of dermatomyositis with typical features of punctate porokeratotic keratoderma, both as paraneoplastic presentation of ovarian carcinoma. Its paraneoplastic nature is clearly demonstrated by the clinical improvement observed following tumor excision.

Introduction While the standardization of exploration with photoallergy tests or photopatch testing runs its course in Europe, we have carried out an epidemiological study about the current situation of photoallergy in our country. Material and methods We have gathered the results of photopatch testing in seven hospital centres of Madrid, Cataluna, Galicia and Comunidad Valenciana during the years 2004 and 2005. The exploration has included, at least, the standard set of the Spanish Photobiology Group (GEF), with 16 (photo) allergens, that have been irradiated with 10 J/cm 2 . We have assessed the total number of explored patients, their sex, present, past or unknown relevance of positive photopatch testing, cross reactions, and allergens responsible for photosensitization. Results Of 224 patients explored by photopatch testing, 39.3% show one or more positive tests. Seventyone percent (103) were considered relevant with respect to clinical history, 14 cases (9.6%) were cross reactions, and 28 (19.3%) were considered of unknown relevance. The most prevalent allergens were nonsteroidal antiinflammatory drugs, specially ketoprophen (43 patients), followed by bencydamine (7 patients) and etofenamate (5 patients). The mixture of four sunscreens from the standard set of the GEF only detected 10 of 16 patients with photoallergy to sunscreens. Photopatch testing of unknown relevance was mainly due to antiseptics (fenticlor) and topical antihistamines. Conclusions We propose the modification of the standard set of photoallergens from the GEF, that should include the majority of nonsteroidal antiinflammatory drugs and sunscreens available in Spain. Ketoprophen continues to be the most frequent photoallergen in our country. It is also important for the cross sensitizations that may present. Sunscreens should be explored separately and not in form of a mixture.