Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years. / Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.

First-line treatment in lymphomatoid papulosis: a retrospective multicentre study.

BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical steroids, methotrexate and phototherapy were the most common first-line treatments, prescribed for 35%, 20% and 14% of the patients, respectively. Complete response (CR) was achieved in 48% of treated patients. Eczematous lesions significantly increased relative risk (RR) of not achieving CR (RR = 1.76; 95% CI 1.16-2.11). Overall median time to CR was 10 months (95% CI 6-13 months), and 78% of complete responders showed cutaneous relapse; both results were similar for all treatment groups (P > 0.05). Overall estimated median disease-free survival (DFS) was 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; P < 0.03). Having the Type A LyP variant (RR = 2.04; 95% CI 0.96-4.30) and receiving a first-line treatment other than phototherapy (RR = 5.33; 95% CI 0.84-33.89) were significantly associated with cutaneous early relapse. Of the 252 patients, 31 (13%) had associated mycosis fungoides unrelated to therapeutic approach, type of LyP or T-cell receptor clonality. CONCLUSIONS: Current epidemiological, clinical and pathological data support previous results. Topical steroids, phototherapy and methotrexate are the most frequently prescribed first-line treatments. Although CR and cutaneous relapse rates do not differ between them, phototherapy achieves a longer DFS. Presence of Type A LyP and use of topical steroid or methotrexate were associated with an increased risk of early relapse.

Recomendaciones de expertos para el tratamiento de la psoriasis en situaciones especiales / Expert recommendations on treating psoriasis in special circumstances

INTRODUCCIÓN Y OBJETIVOS: Existe gran cantidad de información sobre la terapia sistémica y biológica de la psoriasis moderada-grave. Sin embargo, pueden identificarse numerosas situaciones clínicas concretas en las que la evidencia clínica es controvertida y donde resulta útil la opinión consensuada de los expertos. MATERIAL Y MÉTODOS: Un comité científico revisó, de forma sistemática, la bibliografía disponible en 5 escenarios clínicos. En aquellas cuestiones en las que la evidencia era controvertida se llevó a cabo un cuestionario on line según la metodología Delphi, realizado por dermatólogos con experiencia en el manejo de la psoriasis moderada-grave. RESULTADOS: El cuestionario recogió opiniones de 23 dermatólogos y se alcanzó el consenso en 37 de las 66 aseveraciones propuestas (56%). Los resultados permitieron consensuar propuestas en diversas situaciones clínicas, aun cuando la evidencia no fuese firme. Así, tanto el tratamiento intermitente como la desintensificación se consideraron estrategias adecuadas en la optimización de la terapia biológica y en la reducción de costes. La determinación de niveles de fármaco y de anticuerpos antifármaco debería incluirse rutinariamente en el seguimiento de los pacientes psoriásicos tratados con terapia biológica. La coexistencia de artropatía psoriásica y de antecedentes cardiovasculares condiciona la elección de la terapia biológica, prefiriéndose los fármacos anti-TNF alfa como primera elección. En pacientes embarazadas o con deseos de gestación la evaluación personalizada, la gravedad de la psoriasis y la vida media del fármaco son factores relevantes en la toma de decisiones. CONCLUSIONES: La combinación de una revisión sistemática de la literatura y la discusión y opinión estructurada de los expertos permite realizar propuestas para situaciones clínicas concretas

INTRODUCTION AND OBJECTIVES: A great amount of information on systemic and biologic therapies for moderate to severe psoriasis is now available. However, applying the evidence in numerous clinical scenarios has engendered debate; under these circumstances, the consensus of experts is useful. MATERIAL AND METHODS: A scientific committee systematically reviewed the literature relevant to 5 clinical scenarios. An online Delphi survey of dermatologists with experience treating moderate to severe psoriasis was then carried out in order to shed light on questions that remained unresolved by the available evidence. RESULTS: Twenty-three dermatologists responded to the survey and consensus was reached on 37 (56%) of the 66 statements proposed. These results led to consensus on various clinical situations even though firm evidence was lacking. Thus, intermittent therapeutic regimens and strategies for reducing the intensity of treatment are considered appropriate for optimizing biologic treatment and reducing costs. The measurement of drug and antidrug antibody levels should be included routinely when following patients on biologics to treat psoriasis. Concomitant psoriatic arthritis or a history of cardiovascular conditions will influence the choice of biologic; in these situations, an agent with anti-tumor necrosis factor properties will be preferred. Tailored management is important when the patient is pregnant or intends to conceive; drug half-life and disease severity are important factors to take into consideration in these scenarios. CONCLUSIONS: A combination of systematic review of the literature and structured discussion of expert opinion facilitates decision-making in specific clinical scenarios

Expert recommendations on treating psoriasis in special circumstances.

INTRODUCTION AND OBJECTIVES: A great amount of information on systemic and biologic therapies for moderate to severe psoriasis is now available. However, applying the evidence in numerous clinical scenarios has engendered debate; under these circumstances, the consensus of experts is useful. MATERIAL AND METHODS: A scientific committee systematically reviewed the literature relevant to 5 clinical scenarios. An online Delphi survey of dermatologists with experience treating moderate to severe psoriasis was then carried out in order to shed light on questions that remained unresolved by the available evidence. RESULTS: Twenty-three dermatologists responded to the survey and consensus was reached on 37 (56%) of the 66 statements proposed. These results led to consensus on various clinical situations even though firm evidence was lacking. Thus, intermittent therapeutic regimens and strategies for reducing the intensity of treatment are considered appropriate for optimizing biologic treatment and reducing costs. The measurement of drug and antidrug antibody levels should be included routinely when following patients on biologics to treat psoriasis. Concomitant psoriatic arthritis or a history of cardiovascular conditions will influence the choice of biologic; in these situations, an agent with anti-tumor necrosis factor properties will be preferred. Tailored management is important when the patient is pregnant or intends to conceive; drug half-life and disease severity are important factors to take into consideration in these scenarios. CONCLUSIONS: A combination of systematic review of the literature and structured discussion of expert opinion facilitates decision-making in specific clinical scenarios.

Evaluación clínica y terapéutica de los pacientes con psoriasis moderada o grave en España. Estudio Secuence / Clinical and Therapeutic Evaluation of Patients with Moderate to Severe Psoriasis in Spain: The Secuence Study

Introducción: La evaluación de la gravedad de la psoriasis se considera esencial en el manejo terapéutico óptimo de los pacientes. Objetivos: Descripción de las características clínicas y perfil terapéutico de los pacientes con psoriasis moderada a grave en el ámbito español, evaluando su impacto en la calidad de vida de los pacientes. Material y métodos: Estudio observacional, de corte transversal, realizado en 90 unidades dermatológicas en España en 2009. Se incluyeron 442 pacientes diagnosticados de psoriasis moderada a grave que habían iniciado tratamiento con fármacos sistémicos, fototerapia y/o tratamientos tópicos entre 2004 y 2006. Resultados: Los pacientes con psoriasis más grave presentaron de forma significativa un mayor tiempo de evolución de la enfermedad, mayor prevalencia de enfermedades concomitantes y mayor afectación ungueal, del cuero cabelludo, de las flexuras y de la zona palmo-plantar, así como peor calidad de vida. Durante los 5 años previos al inicio del estudio el 68% de los pacientes recibieron tratamientos sistémicos convencionales, el 39,1% fármacos biológicos y el 22,3% fototerapia. En la actualidad el 57,5% estaban siendo tratados con algún fármaco biológico, el 32,6% recibían fármacos sistémicos convencionales y el 11% fototerapia. Conclusiones: La gravedad de la psoriasis se asoció en nuestro estudio con una marcada afectación de la calidad de vida. Independientemente de la gravedad de la psoriasis, el mayor predictor de mala calidad de vida fue la comorbilidad psiquiátrica. El tiempo medio de permanencia en otros tratamientos, incluyendo sistémicos convencionales o fototerapia, hasta el cambio a un primer biológico fue superior a los 2 años (AU)

Background: Evaluation of disease severity is considered essential in the optimal management of psoriasis. Objectives: To describe the clinical characteristics and therapeutic profile of patients with moderate to severe psoriasis in Spain and to assess the impact of the disease on the patients’quality of life. Materials and methods: This was an observational, cross-sectional study carried out in 90 dermatology units in Spain in 2009. We included 442 patients diagnosed with moderate to severe psoriasis who had started treatment with systemic agents, phototherapy, and/or topical treatments between 2004 and 2006. Results: More severe psoriasis was significantly associated with the following: longer disease duration; higher prevalence of concomitant disease; greater involvement of the nails, scalp, flexures, palms, and soles; and poorer quality of life. In the 5 years before the start of the study,68% of the patients had received conventional systemic treatments, 39.1% biologic agents, and22.3% phototherapy. At present, 57.5% of the patients are being treated with biologic agents,32.6% with conventional systemic treatments, and 11% with phototherapy. Conclusions: Severity of psoriasis was associated with a marked impact on quality of life. Regardless of disease severity, psychiatric comorbidity was the strongest predictor of poor quality of life. On average, patients had received other treatments, such as conventional systemic treatments or phototherapy, for more than 2 years before switching to biologic agents for the first time (AU)

Clinical and therapeutic evaluation of patients with moderate to severe psoriasis in Spain: the secuence study.

BACKGROUND: Evaluation of disease severity is considered essential in the optimal management of psoriasis. OBJECTIVES: To describe the clinical characteristics and therapeutic profile of patients with moderate to severe psoriasis in Spain and to assess the impact of the disease on the patients' quality of life. MATERIALS AND METHODS: This was an observational, cross-sectional study carried out in 90 dermatology units in Spain in 2009. We included 442 patients diagnosed with moderate to severe psoriasis who had started treatment with systemic agents, phototherapy, and/or topical treatments between 2004 and 2006. RESULTS: More severe psoriasis was significantly associated with the following: longer disease duration; higher prevalence of concomitant disease; greater involvement of the nails, scalp, flexures, palms, and soles; and poorer quality of life. In the 5 years before the start of the study, 68% of the patients had received conventional systemic treatments, 39.1% biologic agents, and 22.3% phototherapy. At present, 57.5% of the patients are being treated with biologic agents, 32.6% with conventional systemic treatments, and 11% with phototherapy. CONCLUSIONS: Severity of psoriasis was associated with a marked impact on quality of life. Regardless of disease severity, psychiatric comorbidity was the strongest predictor of poor quality of life. On average, patients had received other treatments, such as conventional systemic treatments or phototherapy, for more than 2 years before switching to biologic agents for the first time.

Clinical and Therapeutic Evaluation of Patients with Moderate to Severe Psoriasis in Spain: The Secuence Study. / Evaluación clínica y terapéutica de los pacientes con psoriasis moderada o grave en España. Estudio Secuence.

BACKGROUND: Evaluation of disease severity is considered essential in the optimal management of psoriasis. OBJECTIVES: To describe the clinical characteristics and therapeutic profile of patients with moderate to severe psoriasis in Spain and to assess the impact of the disease on the patients' quality of life. MATERIALS AND METHODS: This was an observational, cross-sectional study carried out in 90 dermatology units in Spain in 2009. We included 442 patients diagnosed with moderate to severe psoriasis who had started treatment with systemic agents, phototherapy, and/or topical treatments between 2004 and 2006. RESULTS: More severe psoriasis was significantly associated with the following: longer disease duration; higher prevalence of concomitant disease; greater involvement of the nails, scalp, flexures, palms, and soles; and poorer quality of life. In the 5 years before the start of the study, 68% of the patients had received conventional systemic treatments, 39.1% biologic agents, and 22.3% phototherapy. At present, 57.5% of the patients are being treated with biologic agents, 32.6% with conventional systemic treatments, and 11% with phototherapy. CONCLUSIONS: Severity of psoriasis was associated with a marked impact on quality of life. Regardless of disease severity, psychiatric comorbidity was the strongest predictor of poor quality of life. On average, patients had received other treatments, such as conventional systemic treatments or phototherapy, for more than 2 years before switching to biologic agents for the first time.

[Autologous hematopoietic stem cell transplantation followed by oral bexarotene in a patient with advanced mycosis fungoides]. / Trasplante autólogo de progenitores hematopoyéticos seguido de bexaroteno oral en paciente con micosis fungoide avanzada.

We describe the case of a 17-year-old patient with rapidly progressing and aggressive mycosis fungoides, with multiple cutaneous tumors and large cell transformation. She was initially treated with 3 cycles of high-dose chemotherapy with mega-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) without response, leading to the decision to undertake autologous hematopoietic stem cell transplantation. Partial remission of the disease was achieved with this treatment and subsequent introduction of oral bexarotene led to complete remission, which has been maintained for more than 3 years with good tolerance of oral therapy. We discuss the advantages and disadvantages of autologous hematopoietic stem cell transplantation and the use of oral bexarotene.