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.

Urgencias dermatológicas: estudio transversal de un hospital de complejidad intermedia / Dermatological emergencies: a cross-sectional study of a hospital of intermediate complexity

OBJETIVO: Conocer si la introducción de criterios de derivación en la urgencia dermatológica modifica el número de pacientes y las patologías derivadas desde el servicio de Urgencias generales, así como el perfil del paciente que hace uso de este servicio. MATERIAL Y MÉTODOS: Estudio observacional que incluyó de forma consecutiva a los pacientes vistos en Urgencias de Dermatología del Hospital Príncipe de Asturias en un periodo de 31 días de 2015, de marzo a mayo, y en el mismo periodo en 2016. Como variables se recogieron sexo, edad, tiempo de evolución, día de la semana, pruebas complementarias, procedencia, motivo de consulta, diagnóstico y grupo, así como el cumplimiento de los criterios, el destino y si tenía cita previa. RESULTADOS: En 2015 acudieron 525 pacientes y 371 en 2016 (p < 0,001). Fueron atendidos 896 pacientes en total (14,45 pacientes/día); el 55,7% eran mujeres y el 44,3% varones. Se realizaron 121 diagnósticos diferentes, siendo los más frecuentes: otras dermatitis, toxicodermias y dermatitis atópica. Tan solo 32 enfermedades suponen el 70% de los diagnósticos. El 51,6% no cumplió los criterios de derivación urgente. CONCLUSIONES: La introducción de criterios de derivación ha permitido reducir el volumen de pacientes en un 30% en el año 2016. Otras dermatitis y las toxicodermias fueron los principales diagnósticos en ambos grupos. El perfil del paciente coincide con el descrito en la literatura

OBJECTIVE: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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

[Dermatological emergencies: a cross-sectional study of a hospital of intermediate complexity]. / Urgencias dermatológicas: estudio transversal de un hospital de complejidad intermedia.

OBJECTIVE: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Registro de linfomas cutáneos primarios de la AEDV: primer año de funcionamiento / The First Year of the AEVD Primary Cutaneous Lymphoma Registry

ANTECEDENTES Y OBJETIVO: Los linfomas primarios cutáneos son enfermedades poco frecuentes. Este artículo describe el Registro de linfomas cutáneos primarios de la AEDV y sus primeros resultados. PACIENTES Y MÉTODOS: Registro de enfermedad de pacientes con linfomas cutáneos primarios. Los centros participantes recogieron datos prospectivamente de todos los pacientes, incluyendo datos del diagnóstico, de los tratamientos, de las pruebas realizadas y del estado actual del paciente. Se realizó un análisis descriptivo. RESULTADOS: En diciembre del 2017 el registro tenía datos de 639 pacientes pertenecientes a 16 hospitales universitarios. Un 60% eran hombres y los diagnósticos, por orden de frecuencia, fueron: micosis fungoide/síndrome de Sézary (MF/SS) (348 casos; 55%), linfoma cutáneo primario de células B (LCCB) (184; 29%), trastorno linfoproliferativo de células T CD30+ (LTCD30+) (70; 11%) y otro tipo de linfoma T (OLT) (37; 5%). El número de casos incidentes recogidos durante el primer año fue de 105 (16,5%). En los pacientes con MF/SS, el diagnóstico más frecuente fue MF clásica (77,3%). La mitad de estos casos se diagnosticaron en estadio IA. La mayoría de los pacientes estaban en remisión parcial (32,5%) o enfermedad estable (33,1%). Los tratamientos más usados fueron los corticoides tópicos (90,8%) seguidos de fototerapia. En los pacientes con LCCB el diagnóstico más frecuente fue el linfoma de la zona marginal (50%). Casi todos los pacientes tuvieron afectación exclusivamente cutánea y casi la mitad fue T1a. La mayoría (76,1%) estaba en remisión completa. Los tratamientos más utilizados fueron la cirugía (55,4%) y la radioterapia (41,9%). En los pacientes con LTCD30+, el diagnóstico más frecuente fue la papulosis linfomatoide (68,6%). La mayoría fueron clasificados T3b (31,4%). La mitad de los casos estaban en remisión completa. Los tratamientos más frecuentes fueron los esteroides tópicos (68,6%), seguidos de la quimioterapia sistémica (32,9%). CONCLUSIÓN: Las características del paciente con linfoma cutáneo primario en España no difieren de otras series descritas en la literatura. El registro facilitará al grupo de linfomas de la AEDV realizar investigación clínica

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/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ 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 coricosteroids (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 papulosis (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

The First Year of the AEVD Primary Cutaneous Lymphoma Registry. / Registro de linfomas cutáneos primarios de la AEDV: primer año de funcionamiento.

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/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ 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 papulosis (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.

Eritema periorbitario en «ojos de mapache»: clave diagnóstica de lupus neonatal / Periorbital "raccoon-eye" erythema: diagnostic key for neonatal lupus

El lupus eritematoso neonatal es una dermatosis mediada por anticuerpos que se adquiere por vía transplacentaria. Puede afectar a la piel o al corazón, y en el 10% de los casos afecta a ambos órganos. Las lesiones cutáneas remiten espontáneamente, mientras que las cardiacas persisten y pueden llegar a ser mortales (10% de los casos). El eritema periocular en «ojos de mapache» es una forma de presentación característica cuando existe afectación cutánea. Exponemos el caso de un varón de 2 meses de edad con lesiones muy representativas de esta entidad, a fin de facilitar su reconocimiento y que su diagnóstico no pase inadvertido. Gracias a la sospecha clínica, se podrá orientar la anamnesis tanto en la madre como en el lactante y se realizarán los estudios complementarios pertinentes de autoinmunidad y electrocardiografía, que confirmarán el diagnóstico y descartarán una posible afectación cardiaca (AU)

Neonatal lupus erythematosus is an antibody-mediated dermatosis that is acquired transplacentally. Skin and heart are the most frequently affected organs; in 10% of cases, both are involved. Cutaneous lesions disappear spontaneously whereas heart involvement persists without improvement and is lethal (in 10% of cases). Periorbital "raccoon-eye" erythema is a characteristic form of presentation when skin involvement is present. We report the case of a 2-month-old male with very representative le-sions of this entity, in order to facilitate its recognition. Due to clinical suspect, the anamnesis can be oriented in both the mother and the infant and the pertinent complementary studies of autoimmunity and electrocardiography will be carried out, which will confirm the diagnosis and rule out a possible cardiac involvement (AU)

Porocarcinoma ecrino: aspectos demográficos, clínicos, histopatológicos y terapéuticos en una serie de 7 pacientes / Eccrine Porocarcinoma: Patient Characteristics, Clinical and Histopathologic Features, and Treatment in 7 Cases

El porocarcinoma ecrino es un tumor anexial cutáneo maligno poco frecuente, que deriva de la porción ductal de las glándulas sudoríparas. Predomina en pacientes de edad avanzada, pudiendo adoptar diversas presentaciones clínicas. El estudio histológico confirma el diagnóstico y establece factores pronósticos relevantes a la hora de decidir el tratamiento. El curso evolutivo es lento, pero su pronóstico es sombrío cuando aparecen metástasis linfáticas o viscerales. Presentamos 7 pacientes diagnosticados de porocarcinoma ecrino, y describimos los aspectos epidemiológicos, clínicos, histopatológicos y los datos relacionados con el tratamiento de cada uno de ellos. Comparamos los datos obtenidos con las mayores series de casos publicadas, obteniendo similares resultados. Ante la ausencia de algoritmos terapéuticos protocolizados se propone un esquema de tratamiento basado en la literatura revisada y la experiencia personal con nuestra serie, que tiene como ejes centrales los factores pronósticos histológicos y la biopsia selectiva de ganglio centinela (AU)

Eccrine porocarcinoma is a rare, malignant cutaneous adnexal tumor that arises from the ducts of sweat glands. Found mainly in patients of advanced age, this tumor has diverse clinical presentations. Histology confirms the diagnosis, detects features relevant to prognosis, and guides treatment. Growth is slow, but the prognosis is poor if the tumor metastasizes to lymph nodes or visceral organs. We report 7 cases of eccrine porocarcinoma, describing patient characteristics, the clinical and histopathologic features of the tumors, and treatments used. Our observations were similar to those of other published case series. Given the lack of therapeutic algorithms or protocols for this carcinoma, we propose a decision-making schema based on our review of the literature and our experience with this case series. The algorithm centers on sentinel lymph node biopsy and histologic features (AU)

Eccrine Porocarcinoma: Patient Characteristics, Clinical and Histopathologic Features, and Treatment in 7 Cases. / Porocarcinoma ecrino: aspectos demográficos, clínicos, histopatológicos y terapéuticos en una serie de 7 pacientes.

Eccrine porocarcinoma is a rare, malignant cutaneous adnexal tumor that arises from the ducts of sweat glands. Found mainly in patients of advanced age, this tumor has diverse clinical presentations. Histology confirms the diagnosis, detects features relevant to prognosis, and guides treatment. Growth is slow, but the prognosis is poor if the tumor metastasizes to lymph nodes or visceral organs. We report 7 cases of eccrine porocarcinoma, describing patient characteristics, the clinical and histopathologic features of the tumors, and treatments used. Our observations were similar to those of other published case series. Given the lack of therapeutic algorithms or protocols for this carcinoma, we propose a decision-making schema based on our review of the literature and our experience with this case series. The algorithm centers on sentinel lymph node biopsy and histologic features.

Pápulas faciales: un signo de alarma / Facial papules: a warning sign

No disponible

Glándulas de Fordyce en cuerpo del pene: un reto diagnóstico / Fordyce glands in the shaft of the penis: a diagnostic challenge

No disponible