Asunto(s)
Pustulosis Exantematosa Generalizada Aguda/patología , Piel/patología , Síndrome de Stevens-Johnson/patología , Pustulosis Exantematosa Generalizada Aguda/inducido químicamente , Pustulosis Exantematosa Generalizada Aguda/tratamiento farmacológico , Administración Oral , Adulto , Anticonvulsivantes/efectos adversos , Biopsia , Esquema de Medicación , Glucocorticoides/administración & dosificación , Humanos , Lamotrigina , Masculino , Valor Predictivo de las Pruebas , Prednisolona/administración & dosificación , Piel/efectos de los fármacos , Síndrome de Stevens-Johnson/tratamiento farmacológico , Síndrome de Stevens-Johnson/etiología , Factores de Tiempo , Resultado del Tratamiento , Triazinas/efectos adversosRESUMEN
We describe a case of CD34-positive infantile myofibromatosis with hemangiopericytoma-like pattern. A 2-day-old Japanese boy presented with multiple hemispherical nodules on the extremities and back. There was a biphasic histological growth in the dermis, accompanied by a hemangiopericytoma-like pattern with antler-like branching vessels. Tumor cells were oval to spindle-shaped myoid cells with bland appearance. Immunohistochemically, vimentin, calponin and CD34 were positive, while α-smooth muscle actin, h-caldesmon, HHF35 and desmin were negative. Although CD34 was positive, the present case could be diagnosed as infantile myofibromatosis. Myopericytoma, myofibroma/myofibromatosis, glomus tumor, glomangiopericytoma and angioleiomyoma share a continuous spectrum of benign hemangiopericytoma-like pattern tumors. Myofibroma/myofibromatosis is nearly included in myopericytoma among pericytic (perivascular) tumors, and could be positive for CD34. Several immunohistochemical panels of smooth muscle markers are needed for the diagnosis of pericytic (perivascular) tumors.