Abstract

Histiocytoses are rare disorders characterized by the accumulation of dendritic cells or macrophages in various
organs, with a predilection for the skin and bone (1). Its clinical behavior varies from mild to life-threatening
forms(2). The first classification of histiocytosis in 1987 (3) consisted of three categories: Langerhans cell (LCH),
non-LC-related (HCNL), and malignant histiocytosis (MH). These inflammatory neoplasms can present in limited,
diffuse forms or with multiorgan involvement (4), and manifest in multiple ways and mimic other diseases, which
is why they represent a diagnostic challenge for the clinic.