RESUMEN
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
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.