Langerhans cell histiocytosis LCH is a proliferative disease of histiocyte-like cells that generally affects children. Immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. Langerhans cell histiocytosis LCH is easy to be misdiagnosed because of its various clinic features and laboratory results. This research focused on the clinicopathological, histopathological, immunohistochemical and other features of LCH and aimed to analyze LCH clinical features for improving diagnosis and decreasing misdiagnosis rate. A case of rare adult LCH was reported and the clinicopathological features were summarized by literature review. The multifocal form of this case includes diabetes insipidus, exophthalmos and mucocutaneous lesions in axillae and anogenital regions, such as infiltrated nodules, extensive coalescing, scaling, crusted papules and ulcerated plaques.
Adult-Type Langerhans Cell Histiocytosis: Minimal Treatment for Maximal Results
Langerhans cell histiocytosis in adults: a case report and review of the literature
Correspondence: John C. Adult-type Langerhans cell histiocytosis ALCH is characterized as a group of disorders associated with abnormal spread and proliferation of dendritic cells of the epidermis. The disease primarily affects children aged 1 to 4 years. Systemic involvement has been noted in adults, particularly in the lungs. Because of a low prevalence among adults, ALCH is difficult to recognize and diagnose, and the uncertainty of the pathogenesis of ALCH limits treatment alternatives.
Langerhans cell histiocytosis LCH in adults is a rare disorder that occurs when the body produces too many Langerhans cells histiocytes , which are a type of white blood cell that helps fight infection. While Langerhans cells are found in normal, healthy people, there is an over-production and build-up of these cells which can lead to organ damage in adults with LCH. Although some forms of this disease were first described over a century ago, it has only been in recent years that LCH has received more attention, especially in adults. The name for this disease has evolved over the years, as new information has been discovered.
A patient may have very limited involvement in one body system or widespread involvement in several different sites and systems. It is also possible to have LCH in a particular location without symptoms. The following symptoms may indicate disease involvement but are not diagnostic of LCH. This disease varies greatly from patient to patient, and some but not all of these symptoms may be present:. Many adults experience severe and sometimes overwhelming pain associated with this disease.