Improvements in the Management of Patients With Generalized Pustular Psoriasis in Spain: Recommendations From a Group of Experts. / Mejoras en la atención al paciente con psoriasis pustulosa generalizada en España: recomendaciones de un grupo de expertos.

This consensus document analyzed the management and emotional journey of patients with GPP (generalized pustular psoriasis), and the desirable course of the disease while detecting critical points and translating them into needs and recommendations. This project was conducted in 3 phases with participation from an advisory committee (n=8), an expert panel (n=15) and patients with GPP (n=6). The patients' disease progression was heterogeneous due to disease variations, different health care models implemented and available resources, and the lack of diagnostic and treatment guidelines. A total of 45 different recommendations have been made to optimize management and address the emotional component of these patients. Five of them stand out for their impact and viability. Therefore, a roadmap of priorities has been made generally available to improve the management of patients with GPP.

Pharmacogenetic biomarkers for secukinumab response in psoriasis patients in real-life clinical practice.

BACKGROUND: Prediction of the response to a biological treatment in psoriasis patients would allow efficient treatment allocation. OBJECTIVE: To identify polymorphisms associated with secukinumab response in psoriasis patients in a daily practice setting. METHODS: We studied 180 SNPs in patients with moderate-to-severe plaque psoriasis recruited from 15 Spanish hospitals. Treatment effectiveness was evaluated by absolute PASI ≤3 and ≤1 at 6 and 12 months. Individuals were genotyped using a custom Taqman array. Multiple logistic regression models were generated. Sensitivity, specificity and area under the curve (AUC) were analysed. RESULTS: A total of 173 patients were studied at 6 months, (67% achieved absolute PASI ≤ 3 and 65% PASI ≤ 1) and 162 at 12 months (75% achieved absolute PASI ≤ 3 and 64% PASI ≤ 1). Multivariable analysis showed the association of different sets of SNPs with the response to secukinumab. The model of absolute PASI≤3 at 6 months showed best values of sensitivity and specificity. Four SNPs were associated with the capability of achieving absolute PASI ≤ 3 at 6 months. rs1801274 (FCGR2A), rs2431697 (miR-146a) and rs10484554 (HLCw6) were identified as risk factors for failure to achieve absolute PASI≤3, while rs1051738 (PDE4A) was protective. AUC including these genotypes, weight of patients and history of biological therapy was 0.88 (95% CI 0.83-0.94), with a sensitivity of 48.6% and specificity of 95.7% to discriminate between both phenotypes. CONCLUSION: We have identified a series of polymorphisms associated with the response to secukinumab capable of predicting the potential response/non-response to this drug in patients with plaque psoriasis.

Multidisciplinary Management of the Adverse Effects of Apremilast. / Manejo de los efectos adversos de apremilast desde un abordaje multidisciplinar.

We present a series of general and specific recommendations based on pathophysiologic considerations for managing the most common adverse effects of apremilast that lead to treatment discontinuation: diarrhea, nausea, and headache. The recommendations are based on a review of the literature and the experience of a multidisciplinary team of 14 experts including dermatologists, rheumatologists, neurologists, gastroenterologists, pharmacists, and nurses. We propose a series of simple algorithms that include clinical actions and suggestions for pharmacologic treatment. The adverse effects of apremilast can be managed from a multidisciplinary approach. The purpose of optimizing management is to bring clinical benefits to patients.

Description of Patients Treated with Biologic Drugs as First-Line Systemic Therapy in the BIOBADADERM Registry Between 2008 and 2016. / Descripción de los pacientes que reciben biológicos como primer tratamiento sistémico en el registro BIOBADADERM durante el periodo 2008-2016.

INTRODUCTION AND OBJECTIVES: Biologic drugs are usually prescribed as second-line treatment for psoriasis, that is, after the patient has first been treated with a conventional psoriasis drug. There are, however, cases where, depending on the characteristics of the patient or the judgement of the physician, biologics may be chosen as first-line therapy. No studies to date have analyzed the demographics or clinical characteristics of patients in this setting or the safety profile of the agents used. The main aim of this study was to characterize these aspects of first-line biologic therapy and compare them to those observed for patients receiving biologics as second-line therapy. MATERIAL AND METHOD: We conducted an observational study of 181 patients treated in various centers with a systemic biologic drug as first-line treatment for moderate to severe psoriasis between January 2008 and November 2016. All the patients were registered in the Spanish Registry of Adverse Events Associated with Biologic Drugs in Dermatology. RESULTS: The characteristics of the first- and second-line groups were very similar, although the patients receiving a biologic as first-line treatment for their psoriasis were older. No differences were observed for disease severity (assessed using the PASI) or time to diagnosis. Hypertension, diabetes, and liver disease were all more common in the first-line group. There were no differences between the groups in terms of reasons for drug withdrawal or occurrence of adverse effects. CONCLUSIONS: No major differences were found between patients with psoriasis receiving biologic drugs as first- or second-line therapy, a finding that provides further evidence of the safety of biologic therapy in patients with psoriasis.

Evidence-based guidelines of the spanish psoriasis group on the use of biologic therapy in patients with psoriasis in difficult-to-treat sites (nails, scalp, palms, and soles).

Psoriatic lesions affecting the scalp, nails, palms, and the soles of the feet are described as difficult-to-treat psoriasis and require specific management. Involvement of these sites often has a significant physical and emotional impact on the patient and the lesions are difficult to control with topical treatments owing to inadequate penetration of active ingredients and the poor cosmetic characteristics of the vehicles used. Consequently, when difficult-to-treat sites are involved, psoriasis can be considered severe even though the lesions are not extensive. Scant information is available about the use of biologic therapy in this setting, and published data generally comes from clinical trials of patients who also had moderate to severe extensive lesions or from small case series and isolated case reports. In this article we review the quality of the scientific evidence for the 4 biologic agents currently available in Spain (infliximab, etanercept, adalimumab, and ustekinumab) and report level i evidence for the use of biologics to treat nail psoriasis (level of recommendation A) and a somewhat lower level of evidence in the case of scalp involvement and palmoplantar psoriasis.

Impact of atopic dermatitis on health-related quality of life in Spanish children and adults: the PSEDA study.

BACKGROUND AND OBJECTIVES: Atopic dermatitis (AD) affects both children and adults. The main symptom of this disease is pruritus, which impacts health-related quality of life (HRQOL). The aim of this study was to evaluate the impact of AD on the lives of children and adults with this disease. PATIENTS AND METHODS: This was a multicenter prospective epidemiological study of children (2-17 years) and adults (≥ 18 years) with AD selected from dermatology offices in Spain. The patients had all had AD for at least a year and had no other chronic inflammatory skin diseases. In addition to sociodemographic information, the data collected included the clinical features of AD and the results of patient-centered questionnaires: the Itch Severity Scale (ISS), the Dermatology Life Quality Index (DLQI), and the children's version of this questionnaire (cDLQI). RESULTS: We studied 151 children and 172 adults. The mean (SD) age of the children was 9.4 (4.5) years and 51.7% were boys. In the case of adults, the mean age was 32.3 (13.4) years and 58.7% were women. Among the children, the ISS score increased with disease severity and in the adults, both the frequency and intensity of pruritus increased with disease severity (P<.05 in both cases); 79% of the children and 87.1% of the adults reported difficulty sleeping. In both populations, overall and subscale DLQI and cDLQI scores varied according to disease severity. CONCLUSIONS: AD affects HRQOL in both children and adults and its impact is correlated with the presence and intensity of pruritus.

[Pharmacogenetics I. Concept, history, objectives and areas of study]. / Farmacogenética I. Concepto, historia, objetivos y áreas de estudio.

There is great interindividual variability in the response to drugs, both in effectiveness and toxicity. Different patients respond differently to the same medication as a consequence of genetic and non-genetic factors. Pharmacogenetics represents the study of the individual pharmacological response based on the genotype. Its objective is to optimize treatment in an individual basis, thereby creating an individualized therapy, more safe and efficient, that will allow the clinician to select the correct drug, at the adequate dose, for the right patient. In the first part of this review we discuss several aspects of the relationship between genetics and therapeutic response, as well as the concept, history, objectives and different areas of study of pharmacogenetics.

Sclerodermatous graft-versus-host disease after donor leucocyte infusion.

Leucocyte infusion from the marrow donor is a new therapeutic option to avoid repeated bone marrow transplants in patients with recurrent leukaemia. The treatment is based on the fact that alloreactive donor immune cells may recognize and eliminate residual leukaemia cells. This phenomenon has been called graft-versus-leukaemia. We describe a patient who received donor leucocyte infusion for a relapsed chronic myeloid leukaemia after his second bone marrow transplant and developed sclerodermatous graft-versus-host disease.

Recall phenomenon with the unusual presence of eccrine squamous syringometaplasia.

The recall phenomenon is an inflammatory reaction limited to a previously X-irradiated field when the patient is treated months or years later with certain drugs. Only a few cases have been reported in dermatological journals. We report a patient with lymphoma who was treated with low-dose abdominal irradiation and high-dose irradiation to the knees and who, 2 months later, when chemotherapy was started, developed a pronounced inflammatory reaction limited to the areas treated with high-dose irradiation. A skin biopsy specimen showed features of radiation damage, marked epidermal changes and extensive eccrine squamous syringometaplasia. This case of the recall phenomenon is of interest because we have found that there is apparently a radiation dose threshold for this event, and because this is the first report of its association with eccrine squamous syringometaplasia.

Lichen amyloidosus and human immunodeficiency virus infection.

A case of lichen amyloidosus in an HIV-infected patient is presented. To our knowledge, the association between lichen amyloidosus and HIV infection has not been described previously. The possible relationship between both entities is discussed.