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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.

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.