Letterer-Siwe disease

Letterer-Siwe disease is an acute, severe, multisystem form of Langerhans cell histiocytosis characterized by the clonal proliferation and widespread tissue infiltration of pathologic dendritic mononuclear cells Without prompt systemic intervention, this disseminated histiocytic proliferation carries a high risk of rapidly progressive organ dysfunction and significant mortality

Letterer-Siwe disease is an acute, severe, multisystem form of Langerhans cell histiocytosis characterized by the clonal proliferation and widespread tissue infiltration of pathologic dendritic mononuclear cells It primarily affects infants and children younger than two years of age, presenting as a disseminated disorder involving high-risk organ systems Clinically, it manifests with constitutional symptoms such as fever and failure to thrive, alongside a characteristic scaly or purpuric seborrh...

It primarily affects infants and children younger than two years of age, presenting as a disseminated disorder involving high-risk organ systems Clinically, it manifests with constitutional symptoms such as fever and failure to thrive, alongside a characteristic scaly or purpuric seborrheic-like cutaneous eruption, lymphadenopathy, hepatosplenomegaly, cytopenias from bone marrow infiltration, and potential pulmonary or skeletal involvement