Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.

INTRODUCTION: PRP is a rare entity of unknown etiopathogenesis. Lack of clinical practice guidelines makes management challenging for clinicians. OBJECTIVE: To add our experience to the corpus evidence on PRP. METHODS: This was a retrospective, descriptive, and multicentric study of 65 patients with PRP, the largest European case series of patients with PRP ever reported. RESULTS: PRP was more prevalent in men with a mean age of 51 years, yet erythrodermic forms presented in older patients (mean age, 61 years).Six (75%) pediatric patients and 10 (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. However, 26 (68%) erythrodermic patients required biologic therapy as the last and effective therapy for a mean 6.5 months to achieve complete response. CONCLUSION: Our study showed a statistical difference in terms of outcome and response to treatment between children, or patients with limited disease and patients who develop erythroderma.

Unmet Needs in the Management of Moderate-to-Severe Psoriasis in Spain: A Multidimensional Evaluation.

Psoriasis is a chronic, systemic inflammatory disease that affects the skin, with a high impact on patients' quality of life. The aim of this study was to identify and determine the relative importance of unmet needs in the management of moderate-to-severe psoriasis in Spain, from a multi-stakeholder perspective. A mixed method-approach was used to collect information, design a questionnaire and a discrete-choice exercise, and elicit the unmet needs through a multidisciplinary committee composed of 12 experts. A total of 65 unmet needs were identified and categorized into 4 areas: clinical, patient-related, decision-making process, and social. Decision-making process unmet needs were perceived as the most pressing ones, followed by social, clinical and patient-related. Individually, the need to incorporate outcomes that are important to the patients and to have treatments that achieve total clearance with a rapid onset of action and long-term persistence were the most important unmet needs.

Linear Morphea Mimicking Lichen Striatus in Its Early Presentation.

Linear morphea and lichen striatus are distinct conditions that have been linked in only one previous case report. We describe two patients with facial lichen striatus preceding linear morphea at the same site. A possible pathogenic relationship is discussed.

[Reactions to infliximab infusions in dermatologic patients: consensus statement and treatment protocol. Working Group of the Grupo Español de Psoriasis de la Academia Española de Dermatología y Venereología ]. / Reacciones a la infusión de infliximab en pacientes dermatológicos. Grupo de Estudio, Grupo Español de Psoriasis de la Academia Española de Dermatología y Venereología.

Infliximab is a chimeric monoclonal antibody that binds to and blocks tumor necrosis factor alpha and is the most effective biologic agent approved for the treatment of moderate-to-severe psoriasis. It is administered by intravenous infusion, usually in day hospitals on an outpatient basis. The main problem with the administration of infliximab is the possibility of infusion reactions, which may be immediate or delayed; these reactions are related to the immunogenicity of this monoclonal antibody, leading to the production of anti-infliximab antibodies. Infusion reactions to infliximab are not usually anaphylactic (ie, they are not mediated by immunoglobulin E), and re-exposure of the patient using specific protocols to prevent and treat these reactions is therefore possible. The extensive experience in the use of infliximab for the treatment of rheumatic conditions and chronic inflammatory bowel disease has made it possible to develop infusion reaction management protocols; these can be applied to dermatologic patients, who constitute a growing proportion of patients treated with intravenous biological agents. The aim of this review is to draw up a consensus protocol for the treatment of infusion reactions in dermatologic patients treated with infliximab.

Linfoma b cutáneo primario de centro folicular con epidermotropismo / Primary cutaneous follicle center cell lymphoma with epidermotropism

El linfoma cutáneo primario de células del centro folicular (LCPCF) de la clasificación EORTC es el linfoma cutáneo de células B más frecuente. Su presentación clínica es variable, pero por lo general las lesiones se localizan en la cabeza y el cuello o el tronco y tiene un curso indolente, siendo infrecuente la diseminación extracutánea. Histológicamente las lesiones se caracterizan por un infiltrado nodular o difuso formado por centrocitos y ocasionales centroblastos con abundantes células T reactivas en los estadios iniciales que casi siempre respeta la epidermis. Presentamos el caso de un varón de 67 años con una pápula localizada en región cervical, cuyo estudio histopatológico fue diagnóstico de linfoma B cutáneo primario (LCPCF de la clasificación EORTC), destacando la presencia de granulomas y un evidente epidermotropismo de linfocitos B. El estudio de extensión fue negativo y la lesión fue tratada mediante extirpación quirúrgica y radioterapia, sin recidiva hasta la fecha. El epidermotropismo de linfocitos, si bien constituye una característica histopatológica habitual en los linfomas T cutáneos, puede observarse ocasionalmente en los linfomas B cutáneos primarios; para algunos autores la demostración de la estirpe B de los linfocitos epidermotropos es muy sugestiva del diagnóstico de linfoma B cutáneo primario (AU)