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Histiocytosis are caused by pathogenic myeloid cells, and can be classified as Langerhans cell histiocytosis (LCH) and non-LCH. Erdheim-Chester disease (ECD) is a non-LCH, characterized by multi-organ involvement, typical imaging findings, and confirmatory histological studies. A case with multi-organ involvement and histological confirmation is presented.
View Article and Find Full Text PDFAdult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report.
Radiol Case Rep
March 2025
Radiology Department, Hamad Medical Corporation, Doha, Qatar.
Langerhans cell histiocytosis (LCH) is a rare disorder, especially among adults, characterized by abnormal accumulation of dendritic histiocytes in various tissues, presenting as either single- or multi-system disease. In adults, spinal involvement is less common than long bone, while central nervous system manifestations, such as pituitary gland enlargement and stalk thickening, affect about a quarter of adult patients and may lead to significant endocrine disorders. Salivary gland involvement is another extremely rare manifestation of LCH.
View Article and Find Full Text PDFErdheim-Chester disease: Comprehensive insights from genetic mutations to clinical manifestations and therapeutic advances.
Dis Mon
January 2025
Department of Pharmaceutical Sciences, Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow-226025, (U.P.), India. Electronic address:
Erdheim-Chester disease (ECD) is an extremely rare non-Langerhans cell disorder that is believed to include both inflammatory and neoplastic characteristics. It is caused due to genetic mutations in proto-oncogenes like BRAF and MEK, while immunological pathways have an essential role in the onset and progression of the disease. Despite its rarity, ECD poses significant diagnostic and therapeutic challenges due to its heterogeneous clinical presentation and limited understanding of its underlying pathophysiology.
View Article and Find Full Text PDFNon-Langerhans cell histiocytosis in a patient with an acute myelogenous leukemia relapse: A case report.
Int J Dermatol
January 2025
Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon.
Multicentric Reticulohistiocytosis.
Skinmed
January 2025
Department of Dermatology, Temple University Lewis Katz School of Medicine, Philadelphia, PA;
A Caucasian woman in her twenties having asymptomatic papules on the hands for the past 6 months was referred by rheumatology for a skin biopsy. The patient had presented to rheumatologist for arthralgia. On physical examination, multiple, dull red, 2-5-mm papules were observed on her dorsal fingers, with most in the periungual regions.
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