Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is an uncommon hematological disorder affecting infants and young children. It is the condition characterized by uncontrolled stimulation and proliferation of normal antigen presenting cells, Langerhans cells. Because of its relatively low incidence, limited data are available regarding the epidemiology of LCH, with estim...

Generalized eruptive histiocytosis is a benign proliferative disorder of non-Langerhans cells. It is a very rare disease. The disease presents with soft to firm fleshy papules on face, neck and upper trunk. Biopsy is often needed to make the diagnosis because of its rarity and diverse presentation. There is tendency for the disease to regress spontaneously without treatment. Treatment, if any n...

Congenital self-healing reticulohistiocytosis, also known as Hashimoto-Pritzker disease, is a single system Langerhans cell histiocytosis that typically presents in healthy newborns and spontaneously regresses. In the present report, we described a 2-month-old Thai female newborn with multiple hypopigmented flat-topped papules without any internal organ involvement including normal blood cell c...

Monoclonal antibody against histiocytosis X cells (HXCs) was established. The antigen was the cell membrane of HXCs from the submandibular lesion of a 63-year-old man who had been diagnosed as an adult type of histiocytosis X (HX) and whose HXCs had numerous Birbeck granules (BGs). The obtained monoclonal antibody, named MI1, reacted with the antigenic cell membrane of HXC. Immunoblotting showe...

Langerhans' cell histiocytosis (LCH) is one of the 'histiocytosis disorders', as defined by the Histiocyte Society. LCH is an abnormal proliferation and dissemination of clonal Langerhans' cells in which they accumulate, along with other inflammatory cells, and form tissue granulomas in different organs. It is named after the appearance of the cells, which resemble the normal dendritic cells fo...

Langerhans cell histiocytosis (LCH) is an infrequent disorder complex. It is a disease of myeloid dendritic cells, lymphocytes, and macrophages mixed with eosinophils and neutrophils [1]. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, and organ dysfunction. Langerhaus cell histocytosis occurs at all ages from infancy up to senility with the ...

Langerhans cell histiocytosis is a rare disease of unknown cause, characterized by the proliferation of histiocytic cells (Langerhans cells), that can sometimes be especially difficult to diagnose due to its wide clinical spectrum, ranging from a single lesion to a multisystemic disorder. Appropriate disease staging is fundamental, since treatment depends upon the severity of the disease, and i...

Sinus histiocytosis with massive lymphadenopathy (SHML) is a distinct benign clinicopathological entity, characterized by painless enlargement of lymph nodes due to sinus histiocytosis. Here, we report a case of SHML with diffuse large B-cell lymphoma. A 64-year-old man was admitted to our hospital because of fever. He presented with enlargement of a small cervical lymph node and huge abdominal...

Received: 18 April 2008 Accepted: 8 October 2008 Abstract: Langerhans cell histiocytosis is a relatively rare unique disease process characterized by an abnormal proliferation of immature dendritic cells usually affecting children and young adults. Jaws are involved in less than 10% of children with the disease while mandibular involvement in young children is uncommon and bilateral affection i...

Accepted 29 March 1996 In 1953, Lichtenstein introduced the unifying term 'histiocytosis X' to embrace several previous eponyms, including eosinophilic granuloma, Letterer-Siwe disease and Hand-Schuller-Christian disease.' In 1987, a more prominent designation, Langerhans cell histiocytosis (LCH), was recommended to replace histiocytosis X.' The term is more appropriate as it reflects the fact ...