RESUMO
We report a case of congenital multisystem Langerhans cell histiocytosis with cutaneous and hematopoietic involvement. After the failure of first-line (vinblastine and prednisolone) and second-line (vincristine and cytarabine) therapies, treatment with cobimetinib, a mitogen-activated protein kinase (MEK) inhibitor, led to the remission of disease and a sustained response after 11 months of ongoing treatment. Protein kinase inhibitors targeting BRAF or MEK could represent a promising future therapeutic option, also in children with LCH.
Assuntos
Azetidinas , Histiocitose de Células de Langerhans , Piperidinas , Humanos , Histiocitose de Células de Langerhans/tratamento farmacológico , Histiocitose de Células de Langerhans/congênito , Azetidinas/uso terapêutico , Piperidinas/uso terapêutico , Inibidores de Proteínas Quinases/uso terapêutico , Masculino , Feminino , LactenteRESUMO
When the diagnosis of penile calciphylaxis is suggested, the evolution of the disease is rapidly unfavorable; in this case, a rapid medical treatment must be established to obtain an improvement of the disease to avoid penectomy.