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.

Calcium Channels in Vascular Smooth Muscle.

Calcium (Ca2+) plays a central role in excitation, contraction, transcription, and proliferation of vascular smooth muscle cells (VSMs). Precise regulation of intracellular Ca2+ concentration ([Ca2+]i) is crucial for proper physiological VSM function. Studies over the last several decades have revealed that VSMs express a variety of Ca2+-permeable channels that orchestrate a dynamic, yet finely tuned regulation of [Ca2+]i. In this review, we discuss the major Ca2+-permeable channels expressed in VSM and their contribution to vascular physiology and pathology.

Dermatomiositis del adulto asociada a lipodistrofia / Adult Dermatomyositis Associated With Lipodystrophy

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Study of oral, ear, nose and throat involvement in pemphigus vulgaris by endoscopic examination.

BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune blistering skin disorder characterized by the presence of suprabasal acantholysis and autoantibodies against desmoglein 3. There are two different clinical forms: mucocutaneous (MCPV) and mucosal (MPV). However, little is known about PV lesions in oral, ear, nose and throat (OENT) areas produced by the very dynamic of the anatomical structures involved in the functions of the aerodigestive tract. OBJECTIVES: To investigate the pattern of OENT manifestations in PV, and their relationship with physiological traumatic mechanisms in stratified squamous epithelial structures. METHODS: A prospective analysis of 40 patients diagnosed with MCPV (22 patients) or MPV (18 patients) was carried out at the University Clinic of Navarra. OENT manifestations were evaluated in all patients endoscopically. OENT involvement was divided into anatomical areas. RESULTS: The most frequent symptom was pain, mainly on oral mucosa (87·5%). Buccal mucosa (90%), posterior wall of pharynx (67·5%), upper edge of epiglottis (85%) and nasal vestibule (70%) were the areas most frequently affected in the OENT mucosa. These localizations were related to physiological traumatic mechanisms in polystratified squamous epithelial structures. CONCLUSIONS: OENT endoscopy should be included in the examination of all patients with PV. Knowledge of the most frequent localizations of active lesions on OENT mucosa in PV will help us to interpret more efficiently the findings from OENT endoscopy. Also, information related to traumatic physiological mechanisms on OENT areas must be offered to patients in order to avoid the appearance of new active PV lesions.

Metástasis cerebral de cáncer de endometrio en paciente joven / Brain metastases of endometrial carcinoma in a young woman

Se presenta un caso de metástasis cerebral de cáncer de endometrio precediendo al diagnóstico del tumor primario en una paciente de 43 años, sin factores de riesgo para cáncer de endometrio. Los estudios anatomopatológicos diagnosticaron un adenocarcinoma endometrial pobremente diferenciado, con importantes áreas vasculares demostradas mediante positividad para el factor VIII. Aunque se han descrito metástasis intracraneales en casos de estadios tempranos de la enfermedad o en mujeres sin sospecha de cáncer endometrial, su presentación es excepcional. Cuando encontramos sangrados irregulares a esa edad, no solemos sospechar la existencia de cáncer y es habitual que se intente regularlos con terapia hormonal, lo que puede retrasar el diagnóstico

We report a case of brain metastases preceding diagnosis of the primary tumor in a 43-year-old woman without risk factors for endometrial carcinoma. Histological examination and immunohistochemical studies revealed a poorly differentiated endometrial adenocarcinoma with highly vascular areas identified by factor VIII-positive cells. Although brain metastases have been described early in the course of the disease and in patients without suspected endometrial cancer, such cases are uncommon. In women of this age with bleeding disturbances, endometrial carcinoma is not usually suspected as the primary cause. The disturbance is normally treated with hormone therapy, which can delay diagnosis

[Castleman's disease in the retroperitoneal region]. / Enfermedad de Castleman de localización retroperitoneal.

OBJECTIVE: To describe a case of Castleman's disease presenting as a retroperitoneal mass, with special reference to the differential diagnosis from other retroperitoneal lesions. The histological features, variants, clinical manifestations, etiopathogenesis and treatment of Castleman's disease are reviewed. METHODS: A 64-year-old male presented with voiding symptoms and hypogastric pain. An ultrasound scan showed a 5 x 6 cm mass located behind the bladder and above the prostate, which was confirmed by an abdomino-pelvic CT scan. RESULTS: Retroperitoneal sarcoma was suspected and the mass was resected. The histopathological analysis showed giant lymphoid hyperplasia (vascular hyaline variant of Castleman's disease). CONCLUSIONS: Retroperitoneal Castleman's disease is a lymphoproliferative disorder with two well-defined histological types and a mixed variant. Although this lesion is frequently localized to the mediastinum (71%), extrathoracic lesions have been described. Definitive diagnosis is based on the postoperative pathological findings.