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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.

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.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.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%]).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.

Resumen Objetivo Conocer si la introduccion de criterios de derivacion en la urgencia dermatologica modifica el numero de pacientes y las patologias derivadas desde el servicio de Urgencias generales, asi como el perfil del paciente que hace uso de este servicio. Material y metodos Estudio observacional que incluyo de forma consecutiva a los pacientes vistos en Urgencias de Dermatologia del Hospital Principe de Asturias en un periodo de 31 dias de 2015, de marzo a mayo, y en el mismo periodo en 2016. Como variables se recogieron sexo, edad, tiempo de evolucion, dia de la semana, pruebas complementarias, procedencia, motivo de consulta, diagnostico y grupo, asi como el cumplimiento de los criterios, el destino y si tenia cita previa. Resultados En 2015 acudieron 525 pacientes y 371 en 2016 (p Conclusiones La introduccion de criterios de derivacion ha permitido reducir el volumen de pacientes en un 30% en el ano 2016. Otras dermatitis y las toxicodermias fueron los principales diagnosticos en ambos grupos. El perfil del paciente coincide con el descrito en la literatura.

Resumen Antecedentes y objetivo Los linfomas primarios cutaneos son enfermedades poco frecuentes. Este articulo describe el Registro de linfomas cutaneos primarios de la AEDV y sus primeros resultados. Pacientes y metodos Registro de enfermedad de pacientes con linfomas cutaneos primarios. Los centros participantes recogieron datos prospectivamente de todos los pacientes, incluyendo datos del diagnostico, de los tratamientos, de las pruebas realizadas y del estado actual del paciente. Se realizo un analisis descriptivo. Resultados En diciembre del 2017 el registro tenia datos de 639 pacientes pertenecientes a 16 hospitales universitarios. Un 60% eran hombres y los diagnosticos, por orden de frecuencia, fueron: micosis fungoide/sindrome de Sezary (MF/SS) (348 casos; 55%), linfoma cutaneo primario de celulas B (LCCB) (184; 29%), trastorno linfoproliferativo de celulas T CD30+ (LTCD30+) (70; 11%) y otro tipo de linfoma T (OLT) (37; 5%). El numero de casos incidentes recogidos durante el primer ano fue de 105 (16,5%). En los pacientes con MF/SS, el diagnostico mas frecuente fue MF clasica (77,3%). La mitad de estos casos se diagnosticaron en estadio IA. La mayoria de los pacientes estaban en remision parcial (32,5%) o enfermedad estable (33,1%). Los tratamientos mas usados fueron los corticoides topicos (90,8%) seguidos de fototerapia. En los pacientes con LCCB el diagnostico mas frecuente fue el linfoma de la zona marginal (50%). Casi todos los pacientes tuvieron afectacion exclusivamente cutanea y casi la mitad fue T1a. La mayoria (76,1%) estaba en remision completa. Los tratamientos mas utilizados fueron la cirugia (55,4%) y la radioterapia (41,9%). En los pacientes con LTCD30+, el diagnostico mas frecuente fue la papulosis linfomatoide (68,6%). La mayoria fueron clasificados T3b (31,4%). La mitad de los casos estaban en remision completa. Los tratamientos mas frecuentes fueron los esteroides topicos (68,6%), seguidos de la quimioterapia sistemica (32,9%). Conclusion Las caracteristicas del paciente con linfoma cutaneo primario en Espana no difieren de otras series descritas en la literatura. El registro facilitara al grupo de linfomas de la AEDV realizar investigacion clinica.

Resumen Introduccion El tejido conectivo se distribuye ampliamente por todo el organismo, formando parte de la piel y sus anejos y de las articulaciones. Las enfermedades reumatologicas producen inflamacion como consecuencia de mecanismos autoinmunes, de este tejido conectivo (conectivopatias). La piel es un importante organo diana de estas enfermedades, cuyas manifestaciones son, a menudo, el primer signo que orienta a su diagnostico. Lesiones cutaneas asociadas a conectivopatias A lo largo del texto se exponen las caracteristicas de las siguientes enfermedades y tipos de lesiones. Lesiones asociadas a fotosensibilidad: lupus eritematoso y dermatomiositis; lesiones esclerosas: esclerodermia; sindrome de Raynaud; lesiones urticariales: urticaria cronica, urticaria vasculitis, enfermedad de Still; lesiones nodulares subcutaneas: nodulos reumatoides, nodulos subcutaneos, poliarteritis nodosa; dermatosis neutrofilicas; vasculitis; psoriasis; lesiones en mucosas: aftas orales y enfermedad de Behcet. La clinica cutanea de todas ellas es muy variada. Conclusion Es importante el conocimiento de la sintomatologia cutanea, dado que el diagnostico precoz y la instauracion del tratamiento pueden prevenir futuras complicaciones y mejorar el pronostico.

Background and objective: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. Patients and methods: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. Results: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sezary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous [D30' T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid paputosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). Conclusion: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group. (C) 2018 AEDV. Published by Elsevier Espana, S.L.U. All rights reserved.

To determine if the introduction of some referral criteria in the dermatology emergency department has changed the number of patients referred from the emergency department, as well as the type of diseases, and the profile of the patient who uses this service.A cross-sectional study was conducted on all consecutive patients attended in the emergency department of the Hospital Príncipe de Asturias during a 31-day period from March 2015 to May 2015 and in the same period in 2016. Variables collected were: gender, age, days from the onset, day of the week, complementary diagnostic tests, referral, reason for consultation, diagnosis and group, whether or not they met the criteria, destination, and whether or not they had a previous appointment.A total of 525 patients were seen in dermatology emergency department in 2015, and 371 in 2016 (P.001). Of the 896 patients seen 55.7% were women and 44.3% men (14.45 patients per day). A total of 121 diagnoses were made, with the most common being: other dermatitis, drug reactions, and atopic dermatitis. Only 32 pathologies made up 70% of the diagnoses. More than half (51.6%) did not meet the referral criteria.The introduction of referral criteria has led to a reduction of 30% in the number of patients in 2016. The main diagnoses in both groups were other dermatitis and drug reactions. The profile of the patient is in accordance with the description in the literature.

CD30+ lymphoproliferative disorders are the most common group of cutaneous T-cell lymphomas after mycosis fungoides and its subtypes. This group includes lymphomatoid papulosis and CD30+ anaplastic large-cell lymphoma; these 2 entities are the extremes of a spectrum with numerous intermediate varieties in which it is not possible to establish a clear diagnosis based on clinical and histopathologic criteria. CD30+ lymphoproliferative disorders must be differentiated from other lymphoproliferative diseases with CD30+ cells in the tumor infiltrates, such as mycosis fungoides or Hodgkin disease, and also from other inflammatory conditions or nonhematological neoplasms that can include this cell type, such as pityriasis lichenoides et varioliformis acuta or certain mesenchymal tumors (CD30+ pseudolymphomas). In contrast to their systemic homologues, which arise in the lymph nodes, CD30+ lymphoproliferative disorders generally have a good prognosis. It is very important to exclude the presence of a lymphoma of systemic origin with extralymphatic spread, as the prognosis and treatment are different.

Resumen El segundo grupo mas frecuente de linfomas cutaneos de celulas T son los sindromes linfoproliferativos (SLP) CD30+, por detras del grupo de la micosis fungoide (MF) y sus variantes. Estos engloban la papulosis linfomatoide y los linfomas anaplasicos de celulas grandes CD30+, polos de un espectro que dejan en su zona central casos intermedios, donde no se puede establecer con seguridad un diagnostico en base a criterios clinicos e histopatologicos. Los SLP CD30+ deben diferenciarse de otros procesos linfoproliferativos que pueden presentar celulas CD30+ en sus infiltrados tumorales, como la propia MF o la enfermedad de Hodgkin, y tambien de otras entidades inflamatorias o neoplasias no hematologicas que presenten estas celulas, como es el caso de la pitiriasis liquenoide y varioliforme aguda o determinados tumores mesenquimales («pseudolinfomas CD30+»). En general, el pronostico de estos SLP CD30+ es favorable, lo que les diferencia de sus homologos sistemicos, de origen ganglionar. Resulta muy importante descartar la presencia de linfoma de origen sistemico con afectacion extraganglionar, pues el pronostico y el tratamiento seran diferentes.

CD30+ lymphoproliferative disorders are the most common group of cutaneous T-cell lymphomas after mycosis fungoides and its subtypes. This group includes lymphomatoid papulosis and CD30+ anaplastic large-cell lymphoma; these 2 entities are the extremes of a spectrum with numerous intermediate varieties in which it is not possible to establish a clear diagnosis based on clinical and histopathologic criteria. CD30+ lymphoproliferative disorders must be differentiated from other lymphoproliferative diseases with CD30+ cells in the tumor infiltrates, such as mycosis fungoides or Hodgkin disease, and also from other inflammatory conditions or nonhematological neoplasms that can include this cell type, such as pityriasis lichenoides et varioliformis acuta or certain mesenchymal tumors (CD30+ pseudolymphomas). In contrast to their systemic homologues, which arise in the lymph nodes, CD30+ lymphoproliferative disorders generally have a good prognosis. It is very important to exclude the presence of a lymphoma of systemic origin with extralymphatic spread, as the prognosis and treatment are different.

Granular parakeratosis is a rare entity that results from an acquired disorder of keratinization. Clinically presents as dark erythematous plaques, occasionally pruritic, that usually involve the axilla and other intertriginous areas. The pathology is characteristic and consists of thickening of the stratum corneum with compact parakeratosis and retention of keratohyaline granules, whereas the stratum granulosum is preserved. The etiology is unknown although some factors such as irritating physical or chemical agents have been implicated. Treatment response is variable. We report a new case in a 50-year-old woman with brownish and hyperkeratotic plaques on both axillae, of two years duration, with a compatible pathology that showed a favorable response to tacalcitol.