C group cutaneous and mucocutaneous histiocytoses non langerhans cell histiocytosis of skin and mucosa m group malignant histiocytoses. Histiocyte society revised classification of histiocytoses and neoplasms of the macrophagedendritic cell lineages 3. Non langerhans types of histiocytosis also occur fig. To date, no largescale studies have been done on how often lch occurs in.
Langerhans cell histiocytosis lch is one of the histiocytosis disorders, as defined by the histiocyte society. Mri showed pachymeningeal and subependymal enhancement. Progressive mucinous histiocytosis is a rare, benign, non langerhans cell histiocytosis limited to the skin. In this paper, non langerhans histiocytoses of particular interest for the dermatologist are examined.
Herein, we describe a unique case of multisystem nonlangerhans cell histiocytic proliferation with a fulminant clinical course death occurred within 3 months of presentation that showed prominent hp and extensive involvement of multiple organs, including the. Bilateral thickening of the trigeminal nerve was characterized by low signal intensity on t2weighted images and intense. Langerhans cell histiocytosis lch is a rare histiocytic disorder most commonly characterized by single or multiple osteolytic bone lesions or skin rashes demonstrating infiltration with histiocytes with beanshaped nuclei on biopsy with or without histiocytic infiltration of extraskeletal tissues most notably the skin, lungs, lymph nodes. The non lch consist of a long list of diverse disorders which have been difficult to categorize.
Langerhans cell histiocytosis also called histiocytosis x is a rare disorder that primarily affects children. We report a young woman with primary cerebral nonlangerhans cell histiocytosis of the juvenile xanthogranuloma family. Benign cephalic histiocytosis bch falls into the group of nonlangerhans cell histiocytosis nonlch, which is characterized by a benign course and tendency toward spontaneous remission. Nonlangerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of langerhans cells many manifest cutaneously. This group has generally been divided into langerhans cell histiocytosis lch and nonlangerhans histiocytosis. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other non langerhans cell. Langerhans cell histiocytosis is due to uncontrolled monoclonal proliferation of langerhans cells distinctive cells of monocytemacrophage lineage and should be considered a malignancy although its biological behavior is very variable 1,3. Non langerhans cell histiocytosis nlch, or class ii histiocytosis, represents a broad group of different disorders characterized by the proliferation of histiocytes other than langerhans cell lc table 1481. Diabetes insipidus is the most frequent permanent consequence of langerhans cell histiocytosis, developing in around a quarter of patients. Progressive mucinous histiocytosis is a rare, benign, nonlangerhans cell histiocytosis limited to the skin. Your childs symptoms will depend on how langerhans cell histiocytosis affects their body. We also recognized the correlation between pregnancychildbirth.
In these histiocytosis groups, perhaps the most intriging, controversial and unknown ones are non langerhans histiocytoses. Langerhans cell histiocytosis lch treatment may include observation alone, surgery, radiation therapy, or oral, topical, and intravenous medication. Symptoms range from isolated bone lesions to multisystem disease. Langerhans cell histiocytosis information from the histiocytosis association. Determinants of survival in pulmonary langerhans cell granulomatosis histiocytosis x. To date, there is no standard treatment for this disorder, and more than half of the patients succumb within 3 years. Although langerhans cell histiocytosis lch is a familiar entity to most radiologists and to pediatric radiologists in particular, it is but one of a group of disorders caused by the overproduction of histiocytes, a subtype of white blood cells. It has been proposed that benign cephalic histiocytosis bch may be a variant of micronodular juvenile. Langerhans cell histiocytosis is often diagnosed in childhood, usually between ages 2 and 3, but can appear at any age. Langerhans cells, which help regulate the immune system, are normally found throughout the body, especially in the skin, lymph nodes, spleen, lungs, liver, and bone marrow.
These symptoms can also be caused by other health problems. Langerhans cell histiocytosis in nontwin siblings request pdf. The nonlch consist of a long list of diverse disorders which have been difficult to categorize. Aug 24, 2017 erdheimchester disease ecd is a rare nonlangerhans cell histiocytosis that most commonly affects adults and is driven by a high frequency of mutations in braf, map2k1, and kinases promoting mapk signaling. Intherightclinicalcontext,lesionalcells that are cd1a. Reviews unusual variants of nonlangerhans cell histiocytoses ruggero caputo, md,a angelo valerio marzano, md,a emanuela passoni, md,a and emilio berti, mdb milan, italy histiocytic syndromes represent a large, heterogeneous group of diseases resulting from proliferation of. The nonlch consists of a long list of diverse disorders which have been difficult to categorize. Based on our series of 111 cases of non langerhans cell histiocytoses, we present a new unifying concept for this rare group of disorders. Histiocytosis occurs predominantly in children and young adults see table 20. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for nonlangerhanscell histiocytosis. We report a young woman with primary cerebral non langerhans cell histiocytosis of the juvenile xanthogranuloma family. This group has generally been divided into langerhans cell histiocytosis lch and non langerhans histiocytosis. Like other forms of histiocytosis, nonlangerhans cell histiocytosis tends to cause reddishbrown or reddishyellow bumps in the skin and may affect internal organs such as liver, kidneys, lungs. Langerhans cell histiocytosis is most likely to damage bones, but it can affect many body systems.
Langerhans cell histiocytosis treatment pdqpatient. In langerhans cell histiocytosis, excess immature langerhans cells usually form tumors called. A nonlangerhans cell histiocytosis, benign cephalic histiocytosis is characterized by scattered yellowbrown papules and macules concentrated on face, scalp, neck, and upper trunk that appear in the first 2 years of life, though typically after birth. Langerhans cell histiocytosis lch, secondary dendritic cell. Herein, we report a case of generalized eruptive histiocytosis in a 41yearold woman with peculiar clinical and histological features. Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. Not all children with the disease have the same symptoms.
Multisystem radiology 2008 more than just langerhans cell histiocytosis. Abstract histiocytoses are disorders characterised by inflammation and the accumulation of cells derived from the monocyte and macrophage lineages, which results in tissue damage. The non langerhans cell histiocytoses non lch are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of langerhans cells lcs. Erdheimchester disease ecd is a rare nonlangerhans cell histiocytosis that most commonly affects adults and is driven by a high frequency of mutations in braf, map2k1, and kinases promoting mapk signaling.
Sep 28, 2006 generalized eruptive histiocytosis belongs to cutaneous nonlangerhans cell histiocytoses and is a rare, generalized, selfhealing disorder that usually follows a benign clinical course. Langerhans cell histiocytosis lch is the most common of the histiocytic disorders and occurs when the body accumulates too many immature langerhans cells, a subset of the larger family of cells known as histiocytes. Langerhans cell histiocytosis genetics home reference nih. Aug 28, langerhans cell histiocytosis lch is a rare histiocytic disorder most in erdheimchester disease but not in other nonlangerhans cell. Nov 01, 2005 erdheimchester disease is a rare non langerhans histiocytosis with multisystem involvement. The signs and symptoms of lch depend on where it is in. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. Nonlangerhans types of histiocytosis also occur fig. High prevalence of myeloid neoplasms in adults with non. Based on our series of 111 cases of nonlangerhans cell histiocytoses, we present a new unifying concept for this rare group of disorders.
Class iia histiocytosis involves dermal dendritic cells. C group cutaneous and mucocutaneous histiocytoses nonlangerhans cell histiocytosis of skin and mucosa m group malignant histiocytoses. Bilateral thickening of the trigeminal nerve was characterized by low signal intensity on t2weighted images and intense enhancement on t1weighted images after contrast. Histiocytes and nonlangerhans cell histiocytoses in dermatology. Fulminant multisystem nonlangerhans cell histiocytic. Nonlangerhans cell histiocytosis nonlch is a rare multisystemic disorder thought to be derived from dendritic cells or macrophages. The nonlangerhans cell histiocytoses nonlch are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of langerhans cell. Lch is sonamed for its presumed derivation from the langerhans cells specialized dendritic cells found in the skin and mucosa. Although the exact prevalence of anterior pituitary hormone deficiencies is not known, it is probably high and. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for non langerhans cell histiocytosis.
Langerhans cell histiocytosis is a disorder in which excess immune system cells called langerhans cells build up in the body. Langerhans cell histiocytosis is a rare, multisystem disease that shows a particular predilection for hypothalamopituitary axis involvement. The non langerhans cell histiocytoses non lch are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of langerhans cell. A non langerhans cell histiocytosis, benign cephalic histiocytosis is characterized by scattered yellowbrown papules and macules concentrated on face, scalp, neck, and upper trunk that appear in the first 2 years of life, though typically after birth. Lch is part of a group of syndromes called histiocytoses, which are characterized by an abnormal proliferation of histiocytes an. Request pdf langerhans cell histiocytosis in nontwin siblings langerhans cell histiocytosis lch, which has unknown pathogenesis, can manifest as many kinds of signs and symptoms at any age.
It often damages bones, especially the skull, legs, hips, spine and arms. Langerhans cell histiocytosis radiology reference article. Family history of cancer or having a parent who was exposed to certain chemicals may increase the risk of lch. Lch is part of a group of syndromes called histiocytoses, which are characterized.
Endolymphatic nonlangerhans cell histiocytosis of the. Langerhans cell histiocytosis lch is a rare cancer involving clonal proliferation of langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Histiocyte society categorizes histiocytic disorders into 5 groups. Unusual variants of nonlangerhans cell histiocytoses. Histiocytic disorders are currently identified by their component cells.
Any organ or system of the human body can be affected, but those more frequently involved are the skeleton 80% of cases, the skin 33%, and the pituitary 25%. Noah brown, md langerhans cell histiocytosis lch comprises a wide spectrum of clinical disorders that have in common a proliferation of langerhanstype cells with characteristic morphologic, immunophenotypic, and ultrastructural. We found two major groups regarding the initial symptoms. Management of adult langerhans cell histiocytosis based on. Endocrine manifestations in langerhans cell histiocytosis. Medical management of langerhans cell histiocytosis from. In contrast, nonlangerhans histiocytoses are thought to be derived from the monocytemacrophage lineage. A case of generalized eruptive histiocytosis html acta. The common denominator is the monocyte macrophage, which present with various histologic features probably due to the influence of cytokines. Aug 15, 2019 when found in the lungs, it should be. Apart from bch, the main types of nonlch include juvenile xanthogranuloma, generalized eruptive histiocytoma, and xanthoma disseminatum.
Non langerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of langerhans cells many manifest cutaneously. Noah brown, md langerhans cell histiocytosis lch comprises a wide spectrum of clinical disorders that have in common a proliferation of langerhans type cells with characteristic morphologic, immunophenotypic, and ultrastructural. We report a yearold asymptomatic male smoker with a non specific interstitial infiltrate found in preventive chest x ray examination. Erdheimchester disease is a rare, nonlangerhans cell histiocytosis histologically characterized by multisystemic proliferation of mature histiocytes in a background of. In contrast, non langerhans histiocytoses are thought to be derived from the monocytemacrophage lineage.
Erdheimchester disease is a rare nonlangerhans histiocytosis with multisystem involvement. A person of any age can get histiocytosis, but it is most common in children 1 to 3 years old. Histiocytosis association 332 north broadway pitman, nj 08071 tollfree. Nonlangerhans cell histiocytosis nlch, or class ii histiocytosis, represents a broad group of different disorders characterized by the proliferation of histiocytes other than langerhans cell lc table 1481. 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.
Langerhans cell histiocytosis seattle childrens hospital. Endolymphatic nonlangerhans cell histiocytosis of the larynx. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi and intramedullary spinal. Successful treatment of erdheimchester disease, a non. The non lch consists of a long list of diverse disorders which have been difficult to categorize. Histiocytes and nonlangerhans cell histiocytoses in. The nonlangerhans cell histiocytoses sheila weitzman, mb 1,2 and ronald jaffe, mb, bch 3 introduction histiocytic disorders are currently identi. The nonlch consist of a long list of diverse disorders which have been dif. Ten casesall womenin four families and one sporadic case have been described in. Isolated bone marrow nonlangerhans cell histiocytosis. Treatment can include chemotherapy, surgery, radiation therapy, photodynamic therapy, immunotherapy, and targeted therapy. Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body.
Juvenile xanthogranuloma jxg, erdheimchester disease ecd, and rosaidorfman disease rdd previously were categorized as nonlch disorders. Pdf primary cerebral nonlangerhans cell histiocytosis. Like other forms of histiocytosis, non langerhans cell histiocytosis tends to cause reddishbrown or reddishyellow bumps in the skin and may affect internal organs such as liver, kidneys, lungs. The nonlangerhans cell histiocytoses nonlch are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of langerhans cells lcs. Generalized eruptive histiocytosis belongs to cutaneous nonlangerhans cell histiocytoses and is a rare, generalized, selfhealing disorder that usually follows a benign clinical course. Langerhans cell histiocytosis lch is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal langerhans cells in various organs. Because of the relative rarity of ecd, key clinical features of the disease may not be well defined. Most individuals with adultonset langerhans cell histiocytosis are current or past smokers. Oct 25, 2012 langerhans cell histiocytosis lch is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal langerhans cells in various organs. This proliferation is accompanied by inflammation and. Nonlangerhans cell histiocytosis refers to a family of histiocytosis characterized by the absence of langerhans cells. Get detailed treatment information for lch in this summary for clinicians. Langerhans cell histiocytosis, cranial diabetes insipidus, idiopathic thrombocytopenic, purpura, hypothalamicpituitary axis introduction langerhans cell histiocytosis lch is a rare disease, more so in adults, characterised by proliferation of large mononuclear cells.
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