Langerhans’cell histiocytosis (Histiocytosis X) is a rare disease of unknown cause characterized by oligoclonal proliferation of Langerhans cells. It occurs mostly in children and young adults and involves one or more body systems such as bone, hypothalamus, posterior pituitary gland, lymph nodes, liver or various soft tissues.The diagnosis is always made by a histological approach.We report a ...

Progressive nodular histiocytosis is a rare non-Langerhans histiocytosis. It tends to occur in adult with progressive course that differentiates from other groups of xanthogranuloma. The clinical presentation is characteristic and disfiguring of the face is not uncommon. Extracutaneous involvement and metabolic association were reported in a few cases. Treatment is unsatisfactory and follow-up ...

In this issue of Blood, Hervier et al has identified that cooccurrence of Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) in the same patient is not a rare event. Mixed histiocytosis (MH) highlights existence of a link between distinct groups of histiocytic disorders and suggests presence of a common progenitor cell. Today, histiocytic disorders are classified into 3 group...

UNLABELLED Histiocytic neoplasms are clonal, hematopoietic disorders characterized by an accumulation of abnormal, monocyte-derived dendritic cells or macrophages in Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (non-LCH), respectively. The discovery of BRAF(V600E) mutations in approximately 50% of these patients provided the first molecular therapeutic target in his...

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed ...

Between May 1972 and December 1991, 58 children were seen in the authors' institution for treatment of Langerhans' cell histiocytosis. Of these, 15 children (7 males, 8 females) had biopsy-proven Langerhans' cell histiocytosis with vertebral involvement. The average age at presentation was 6.4 years (range, 0.2-13.3 years). At presentation, 8 patients had involvement of a single vertebra and 4 ...

264 Ann Dermatol Received February 24, 2015, Revised May 7, 2015, Accepted for publication May 7, 2015 Corresponding author: Do Young Kim, Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemoon-gu, Seoul 03722, Korea. Tel: 82-2-2228-2080, Fax: 82-2-393-9157, E-mail: [email protected]. This is an Open Acces...

BACKGROUND Non-Langerhans histiocytosis is a group of inflammatory lymphoproliferative disorders originating from non-clonal expansion of hematopoietic stem cells into cytokine-secreting dendritic cells or macrophages. Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans cell histiocytosis characterized by tissue inflammation and injury caused by macrophage infiltration and histologic...