OBJECTIVE Langerhans cell histiocytosis is an unusual disorder of unknown etiology with heterogeneous clinical behaviors and variable outcomes. It can involve one or more organs or systems, but to our best knowledge, parotid glands involvement in Langerhans cell histiocytosis is extremely rare in English literature. METHOD We report a 13-month-old girl who presented with bilateral parotid swe...

Langerhans cell histiocytosis (LCH), formerly known as histiocytosis-X, manifests by granulomatous lesions consisting of mixed histiocytic and eosinophilic cells. The hallmark of LCH invasion into the CNS is diabetes insipidus, reflecting local infiltration of Langerhans cells into the posterior pituitary or hypothalumus. In five patients who had early onset LCH with no evidence of direct invas...

Langerhans cell histiocytosis (LCH), also designated as histiocytosis-X or eosinophilic granuloma, is an uncommon dendritic cell disorder characterized by the infiltration of abnormally proliferating Langerhans cells into 1 or more organs.1 The clinical presentation of LCH varies depending on the site(s) of involvement and aggressiveness of disease. In children, it may present as LettererSiwe d...

Histiocytosis X, or Langerhans cell histiocytosis (LCH), is a rare disease, with an estimated incidence of 1/200,000 per year in children under 15 years of age. It has a wide clinical spectrum, from single bone involvement (eosinophilic granuloma) to multisystemic disease with organ failure. The treatment of LCH is still controversial. While single system disease may spontaneously recover or re...

In 1973, a 10 year old boy presented with numerous bilateral lung nodules, diagnosed as histiocytosis X by open lung biopsy. The patient was treated with prednisone until 1984. In 1993, he developed severe pain in the neck. A biopsy of the spine revealed the same tumour morphology as was seen in the lung in 1973. Immunohistological examination of the former and present biopsy led to the definit...

Introduction Germline mutations in SLC29A3 result in a range of clinically related, recessive syndromes: H syndrome, pigmented hypertrichosis with insulin-dependent diabetes mellitus (PHID) syndrome, Faisalabad histiocytosis (FHC), and sinus histiocytosis with massive lymphadenopathy (SHML). Main symptoms of these diseases are hyperpigmentation with hypertrichosis, sensorineural deafness, diabe...

Langerhans cell histiocytosis (LCH), the most common histiocytic disorder, is characterized by the accumulation of CD1A(+) /CD207(+) mononuclear phagocytes within granulomatous lesions that can affect nearly all organ systems. Historically, LCH has been presumed to arise from transformed or pathologically activated epidermal dendritic cells called Langerhans cells. However, new evidence support...