We describe a rare case of non-Langerhans histiocytosis, consistent with Erdheim-Chester disease (ECD), which presented with lesions resembling multiple meningiomas. The patient was initially evaluated for migraine headaches. Initial MR imaging demonstrated a parasellar mass and a second mass near the torcula considered to represent meningiomas. Within 1 year, he developed bilateral orbital les...

Lipid histiocytosis of the gallbladder neck lymph node is rarely reported nowadays. Two obese patients presented with gallbladder lithiasis detected on CT scan. The treatment consisted in coelioscopic cholecystectomy. Microscopy revealed subacute/chronic lithiasic cholecystitis and foci of vacuolated cells in the gallbladder neck lymph node. These cells were positive for CD68, CD31, S100 protei...

Relevant information on Langerhans cell histiocytosis development and course in children is presented. The current concepts of disease pathogenesis, principles its severity evaluation, patients stratification into risk groups, as well clinical features are described. Modern approaches to the treatment via targeted therapy summarized analyzed.

LCH: Langerhans cell histiocytosis INTRODUCTION Langerhans cell histiocytosis (LCH) identifies a spectrum of disorders that are classified according to the organs involved (singleor multi-organ disease) and the presence or absence of organ failure. Patients younger than 2 years with a multisystem disease have a significantly higher mortality rate than older children. Nail involvement in LCH is ...

A rare case of adult onset Langerhans' cell histiocytosis associated with dysgerminoma in a 35 year old Chinese woman is reported. The patient had a history of dysgerminoma of left ovary 15 years previously and had undergone surgery followed by radiotherapy and an uneventful recovery. She presented again in March 1994, this time with a left clavicular mass, which was shown histologically to be ...

cells of the mononuclear phagocyte system and the dendritic system(l). Each of the histiocytoses of childhood is characterized by localized or generalized, reactive or neoplastic proliferation of cells similar if not identical to one of these cell types(2). As per Writing Group of the Histiocyte Society(3), various histiocytoses of childhood have been classified into Class I, Class II and Class...

We report a rare case of Langerhans cell histiocytosis involving the sternum. The patient was a 12-year-old girl presenting with anterior chest pain and swelling. Radiographs and computed tomography showed an osteolytic lesion in the sternum. Technetium bone scintigraphy revealed increased uptakes in the sternum, the greater trochanter of the right femur, and the right distal tibia. Incisional ...

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...