Langerhans cell histiocytosis in children

Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male

“…Considering their origin from dendritic cells and key molecular findings, Emile et al proposed a new classification for histiocytic disorders, defining five types: Langerhans, cutaneous and mucocutaneous, malignant, Rosai-Dorfman, hemophagocytic lymphohistiocytosis, and macrophage activation syndrome [8]. Within this schema, LCH belongs in the Langerhans group, along with Erdheim-Chester disease and indeterminate cell histiocytosis, based on its activating mutation in the MAPK pathway [1].…”

“…Historically, LCH is divided into four distinct syndromes: Letterer-Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma, and congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease). In clinical practice, many patients do not fit precisely into a particular entity, frequently exhibiting overlapping manifestations [1]. Clinically, LCH is further categorized based on the extent of disease (single or multisystem) and LCH is primarily a disease of childhood, with an incidence of approximately five per every million children and cutaneous involvement in as many as 40% of cases [1,5].…”

“…In clinical practice, many patients do not fit precisely into a particular entity, frequently exhibiting overlapping manifestations [1]. Clinically, LCH is further categorized based on the extent of disease (single or multisystem) and LCH is primarily a disease of childhood, with an incidence of approximately five per every million children and cutaneous involvement in as many as 40% of cases [1,5]. In comparison, the incidence of LCH in adults is one to two per million [6].…”

“…In comparison, the incidence of LCH in adults is one to two per million [6]. Clinical manifestations of LCH vary; it may present in single or multiple organ systems, single or multiple sites within one specific organ system, or in risk organs, such as the skeletal system, liver, spleen, and skin [1]. Cutaneous presentations range from light brown, yellow-orange, or violaceous macules, papules, or plaques.…”

Isolated Langerhans cell histiocytosis of the spleen

“…[ 13 ] Therefore, current knowledge defined LCH as an inflammatory myeloid neoplasia driven by activating mutations in the mitogen-activated protein kinase (MAPK) pathway. [ 14 ] Due to the diversity of clinical course, the standard treatment for LCH has not been established. Generally speaking, there have 4 basic types of treatments for LCH, including operation, chemotherapy, radiation therapy, and immunotherapy.…”