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Atopic dermatitis; Cutaneous lymphocyte antigen; Skin infections Dermatitis atópica; Antígeno cutáneo de linfocitos; Infecciones de la piel Dermatitis atòpica; Antigen limfòcit cutani; Infeccions de la pell Staphylococcus aureus, memory skin-homing cutaneous lymphocyte-associated antigen (CLA)+ T cells and IL-13 constitute relevant play-ers in atopic dermatitis (AD) pathogenesis.1 Since circulating CLA+ T cells reflect cutaneous abnormalities present in human inflammatory skin diseases,2 an ex vivo coculture model made of purified circu-lating CLA+/− effector and central memory T cells and autologous lesional epidermal cells was established. We show a CLA-dependent production of IL-13 upon activation with staphylococcal enterotoxin B (SEB) that allows the differentiation of the Th2 high and Th2 low groups, with distinct clinical correlations between both groups, within a clinically homogeneous population of adult non-treated moderate-to- severe AD patients.

Resumen Antecedentes El penfigo vulgar y foliaceo son enfermedades ampollares autoinmunes causadas por anticuerpos dirigidos contra la desmogleina 1 y/o 3. El tratamiento clasico son los glucocorticoides orales a dosis altas y otros inmunosupresores como azatioprina o micofenolato. El rituximab (RTX), un anticuerpo monoclonal anti-CD20, se ha mostrado eficaz en el tratamiento de estas entidades. Objetivos Evaluar el grado de respuesta a RTX (respuesta parcial/respuesta completa), el tiempo en alcanzarla, el tiempo hasta la recaida, la evolucion de los anticuerpos antidesmogleina 1/3 y los efectos adversos. Pacientes y metodo Estudio retrospectivo descriptivo unicentrico que incluye todos los pacientes diagnosticados de penfigo vulgar o penfigo foliaceo tratados con RTX y con un seguimiento postratamiento minimo de 6 meses. Resultados Se han incluido 6 pacientes (4 con penfigo vulgar y 2 con penfigo foliaceo) con un seguimiento medio de 84 meses. El total de ciclos de RTX administrados fue de 15, alcanzandose una respuesta completa en 7 ciclos, una respuesta parcial en 6 y sin respuesta en 1. Se objetivo una mejoria en la puntuacion Autoimmune Bullous Skin Disorder Intensity Score tanto en el apartado cutaneo (rango 0-150; media pre/pos-RTX 3,19/1) como en el mucoso objetivo (rango 0-11; media pre/pos-RTX 9,21/1,33). El tiempo hasta la recaida fue de 20,75 meses. Unicamente dos pacientes presentaron efectos adversos inmediatos leves durante la perfusion (cefalea e hipertension arterial). Discusion RTX es un farmaco efectivo y seguro en el tratamiento del penfigo y puede conseguir largos periodos de respuesta sin tratamiento o con minimo tratamiento concomitante, lo que permite evitar EA de la corticoterapia cronica.

The subtypes of chronic urticaria (CU) share a common clinical expression, but phenotypically may show differences. To evaluate sociodemographic and clinical differences in CU phenotypes, including: (1) isolated chronic spontaneous urticaria (CSU); (2) isolated chronic inducible urticaria (CIndU); (3) CSU with concomitant CIndU (CSU-CIndU); (4) CSU with single or multiple episodes; (5) early and late-onset CSU (

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Background The knowledge about chronic spontaneous urticaria (CSU) phenotypes is based on its clinical characteristics, associated comorbidities, course of the disease and its response to the available effective drugs. Genotype expression and its further correlation with CSU phenotypes are still unknown. We describe the cutaneous transcriptome of patients suffering a severely active CSU refractory to antihistamine treatment. Methods Through the bioinformatic analysis of the whole Human Genome with Oligo Microarrays and Quantitative real-time polymerase chain reaction (qPCR), relevant genes expressed in non-lesional [NLS-CSU] and lesional skin [LS-CSU]) and peripheral blood were identified in 20 patients suffering from severely active CSU and 10 healthy controls (HCs) Results From 39 genes differentially expressed in NLS-CSU when compared with HCs, 31 (79.48%) were confirmed by qPCR corresponding to genes involved in epidermal homeostasis and dermal repair. From the analysis comparing LS-CSU with NLS-CSU, a selection of 142 genes was studied with qPCR, and 103 (72.53%) were confirmed. Differentially expressed genes in the phenomenon of wheal development are involved in a variety of biological functions as, epidermal differentiation, intracellular signal function, transcriptional factors cell cycle differentiation, inflammation or coagulation. Differentially expressed genes that uniformly increase or decrease along the skin worsening until the wheal appearance are shown. Conclusion The skin of CSU patients with a severely active disease shows an overall immunological skin involvement showing a peculiar gene profile. This article is protected by copyright. All rights reserved.

The second-generation H1-antihistamines (sgAH) are the first-line symptomatic treatment of patients with chronic spontaneous urticaria (CSU). Up to 50% of the patients will not respond to licensed doses of sgAH. According to the guidelines, the dose of sgAH may be increased up to 4 times the conventional dose. However, even at higher doses, there is a subgroup of patients refractory to the antihistamine treatment. The purpose of this article was to review the different treatment options of antihistamine-refractory CSU patients. This revision examines the available literature for therapies used in chronic urticaria, including omalizumab, ciclosporin A, oral glucocorticoids, leukotriene receptor antagonists, H2 antihistamines, doxepin, dapsone, hydroxychloroquine, phototherapy, methotrexate, mycophenolate mofetil, azathioprine, autohemotherapy, intravenous immunoglobulins and rituximab, between others. After the exhaustive review of the medical literature only few high-quality studies have been identified, mostly for omalizumab. Omalizumab is an anti-immunoglobulin E monoclonal antibody, approved for the treatment of CSU, that has radically changed the management of the patients without good response to sgAH, allowing to reach complete responses in a high percentage of patients. Although actually the therapeutic management of CSU is more effective and safer than before 2014, there is place even for new and more effective treatments. A good number of partial responders and slow responders to omalizumab and a little percentage still of non-responders to available therapies stimulate the development of new drugs that will also be discussed.

Chronic spontaneous urticaria (CSU) is characterized by heterogeneous activity, evolution, associated comorbidities and response to treatment. The aim of this study was to identify prognostic factors in patients with CSU that predict disease course and response to standard treatments. An observational retrospective study was conducted in a cohort of 549 patients with CSU, comparing patients with isolated CSU and those with CSU with concomitant inducible urticaria (CSU-CIndU). The factors associated with a worse prognosis in terms of duration and/or CSU activity and its episodes were: multiple episodes of CSU (19.2% had more than one lifetime episode of CSU), late-onset (63.6% of patients developed first onset of CSU after the age of 45 years), concomitant CIndU (20.2%) and functional serum autoreactivity. Patients with CSU-CIndU required more frequent therapy after 5 years and higher doses of 2nd-generation H1-antihistamines. Of patients with a baseline Urticaria Activity Score 7 (UAS7) between 16 and 42, 84.6% required cyclosporine or omalizumab to achieve symptom control, compared with 15.4% of patients with a baseline UAS7 between 0 and 15 (p?=?0.0013). Baseline CSU activity is the only factor found to be predictive for refractoriness to treatment with H1-antihistamines.

Chronic spontaneous urticaria, also known as chronic idiopathic urticaria or simply chronic urticaria, is a common disorder that has a prevalence in the general population that ranges between 0.5% and 1%. This condition negatively affects the patient's quality of life and has considerable impact on direct and indirect health-related costs. Chronic urticaria is difficult to manage. Nonsedating H1 antihistamines are the first line of therapy, but fewer than 50% of patients experience relief at recommended dosages. Although guidelines call for increasing the dosage when response is inadequate, some patients still do not achieve adequate control of symptoms. New treatment alternatives, with proven efficacy under the standards of evidence-based medical practice, must therefore be developed. © 2012 Elsevier Espana, S.L. and AEDV. All rights reserved.

Keywords: methylchloroisothiazolinone; contact allergy; contact dermatitis; cosmetics; methylisothiazolinone; occupational diseases; patch test; preservatives

BACKGROUND: Data about special phenotypes, natural course, and prognostic variables of patients with acquired cold urticaria (ACU) are scarce. OBJECTIVES: We sought to describe the clinical features and disease course of patients with ACU, with special attention paid to particular phenotypes, and to examine possible parameters that could predict the evolution of the disease. METHODS: This study was a retrospective chart review of 74 patients with ACU who visited a tertiary referral center of urticaria between 2005 and 2015. RESULTS: Fourteen patients (18.9%) presented with life-threatening reactions after cold exposure, and 21 (28.4%) showed negative results after cold stimulation tests (classified as atypical ACU). Nineteen patients (25.7%) achieved complete symptoms resolution at the end of the surveillance period and had no subsequent recurrences. Higher rates of atypical ACU along with a lower likelihood of achieving complete symptom resolution was observed in patients who had an onset of symptoms during childhood (P < .05). In patients with atypical ACU, shorter disease duration and lower doses of antihistamines required for achieving disease control were detected (P < .05). Age at disease onset, symptom severity, and cold urticaria threshold values were found to be related to disease evolution (P < .05). LIMITATIONS: This study was limited by its retrospective nature. CONCLUSIONS: The knowledge of the clinical predictors of the disease evolution along with the clinical features of ACU phenotypes would allow for the establishment of an early and proper therapeutic strategy.

Chronic cold urticaria (ColdU) is a rare disease characterized by mast cell- mediated wheals and angioedema following cold exposure. Second-generation H1-antihistamines, such as rupatadine, are the recommended first-line therapy. As of yet, the effects of rupatadine up-dosing on development of ColdU symptom have only been partially characterized. Two-centre, randomized, double-blind, 3-way crossover, placebo-controlled study in patients with a confirmed ColdU was designed to assess the effects of up-dosing of rupatadine. A total of 23 patients were randomized to receive placebo, rupatadine 20 mg/day, and rupatadine 40 mg/day for 1 week. The primary outcome was change in critical temperature thresholds and critical stimulation time thresholds after treatment. Secondary endpoints included assessment of safety and tolerability of rupatadine. Both 20 and 40 mg rupatadine were highly effective in reducing critical temperature thresholds (p

Introduction The clinical concept of urticaria embraces a heterogeneous group of conditions classified according to their clinical course as acute (lasting less than 6 weeks) or chronic (lasting 6 weeks or more). Chronic urticaria may be either spontaneous or induced. Few tools are available for monitoring the various clinical forms of this disease or for evaluating its impact on quality of life. The recently developed Urticaria Control Test to evaluate disease control is available in German, the original language, and American English. Objective To culturally adapt the long and short versions of the Urticaria Control Test to Castilian Spanish to ensure equivalence between the translated items and those of the original version. Material and methods To translate the Urticaria Control Test we followed the International Society for Pharmacoeconomics and Outcomes Research good practice guidelines, starting with forward translation and moving through back translation and cognitive debriefing steps. Results Three items were modified when the first Spanish version, translated from German, was discussed (cognitive debriefing). The revised translation was then translated back to German and sent to the Urticaria Control Test authors, who modified one item they considered had acquired a different focus through translation. A third Spanish version was then prepared and after minor proofreading changes was considered definitive. Conclusions This study was the first step in making it possible to use the Urticaria Control Test questionnaire in Castilian Spanish. The next step will be to validate the translated questionnaire.

Cold contact urticaria is the second most common subtype of physical urticaria. Cold stimulation standardized tests are mandatory to confirm the diagnosis. The aim of this study is to define the utility of determining thresholds (critical time and temperature) in assessment of the clinical course of typical acquired cold contact urticaria. Nineteen adult patients (10 women and 9 men; mean age 45 years) were included in the study and the diagnosis was confirmed with the ice-cube test and TempTest 3.0. Patients were treated continuously for 1 year with 20 mg/day rupatadine (anti-H1). Thresholds measurements were made before and after treatment. Improvements in temperature and critical time thresholds were found in the study sample, demonstrating the efficacy of continuous treatment with rupatadine. In most cases association with a clinical improvement was found. We propose an algorithm for the management of acquired cold contact urticaria based on these results.

We report the fifth case of epidermal choristoma of the oral cavity in a Caucasian newborn with a congenital melanotic macule on the dorsum of the tongue. Epidermal choristoma is an exceedingly rare and benign condition probably caused by a developmental abnormality. It is identified according to the presence of normal skin in an abnormal location. Histologically it is identified according to areas of stratified epithelium and hyperpigmentation of the basal layer along with cutaneous adnexal structures (hair follicles, sebaceous or sweat glands). The clinical presentation is variable, but most of the cases described presented with a congenital lingual pigmented macule. These lesions should be included within the differential diagnosis of congenital lingual macules and distinguished from other entities such as congenital lingual melanotic macules and melanocytic lesions. Surgical excision is the treatment of choice. Epidermal choristoma is a benign condition that probably is underdiagnosed because it is a new and rare entity, and dermatologists should be aware of it.

AMG-A is Medical Advisor for Uriach Pharma, Genentech, Novartis. Research Grants supported by Intendis – Bayer, Uriach Pharma, Novartis. Educational activities sponsored by Uriach Pharma, Novartis, Genentech, Menarini, Glaxo Smith & Kline, Merck MSD, Almirall Pharma