RESUMEN
Hematodermic CD4+ CD56+ neoplasm with plasmacytoid dendritic cell phenotype is a rare and aggressive neoplasm recently recognized by the WHO-EORTC classification. It generally appears in elderly adults, exceptionally in childhood. We present a 12-year-old girl with severe mental retardation, genetic clinical features and multiple nodular cutaneous lesions on legs and arms. Histologically the nodules showed diffuse dermal infiltrate of medium and small cells and expression of CD4, CD56, CD43, S100 and plasmacytoid dendritic markers: CD123, BDCA-2 under flow cytometry study. Peripheral blood and bone marrow were not involved. Clinical remission of cutaneous lesions was observed after two weeks of acute lymphoblastic leukemia therapy.
Asunto(s)
Biomarcadores de Tumor , Antígenos CD4 , Antígeno CD56 , Linfoma/patología , Neoplasias Cutáneas/patología , Niño , Células Dendríticas/inmunología , Células Dendríticas/patología , Diagnóstico Diferencial , Femenino , Citometría de Flujo , Humanos , Subunidad alfa del Receptor de Interleucina-3/análisis , Células Asesinas Naturales/inmunología , Lectinas Tipo C/análisis , Linfoma/inmunología , Glicoproteínas de Membrana/análisis , Receptores Inmunológicos/análisis , Neoplasias Cutáneas/inmunologíaRESUMEN
A 34-year-old man with an 8-year history of outbreaks of oral and genital ulcers presented with high fever and skin lesions that had developed 21 days earlier, without abdominal pain. Computed tomography revealed intestinal perforation requiring surgery. After treatment with corticosteroids and azathioprine proved to be ineffective, it was switched to adalimumab.
Asunto(s)
Síndrome de Behçet/diagnóstico , Enfermedades del Colon/etiología , Perforación Intestinal/etiología , Adulto , Síndrome de Behçet/complicaciones , Enfermedades del Colon/diagnóstico , Humanos , Perforación Intestinal/diagnóstico , MasculinoRESUMEN
Paciente de sexo masculino de 34 años con antecedente de úlceras orales y genitales que cursaban en brotes de 8 años de evolución, se presenta con fiebre elevada y lesiones en piel durante 21 días, sin dolor abdominal. Se realiza tomografía computarizada, observándose perforación intestinal, por lo que es intervenido quirúrgicamente. Es tratado con corticoides y azatioprina. Luego, por falta de respuesta, se le administra adalimumab (AU)
A 34-year-old man with an 8-year history of outbreaks of oral and genital ulcers presented with high fever and skin lesions that had developed 21 days earlier, without abdominal pain. Computed tomography revealed intestinal perforation requiring surgery. After treatment with corticosteroids and azathioprine proved to be ineffective, it was switched to adalimumab (AU)