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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

1. Langerhans cells represent specific granula-containing dendritic cells which do not have desmosomes, tonofilaments and (pre)melanosomes and which therefore appear as clear cells in the epithelial tissue. 2. They occur in the squamous epithelium, and also in the corium, lymph node and thymus. They account for 1-1,7% of the whole volumen of the epidermis. 3. They do not represent effete melanocytes, but they originate from the mesenchym. Their migration from the dermis into the epidermis, their identity with histiocytosis X cells, their surface receptors as well as function underline the mesenchymal origin. 4. Probably the Langerhans cells regulate the mitosis and differentiation of the keratinocytes. 5. The antigen-presenting and lymphocyte stimulating functions of Langerhans cells as effector cells in allergic contact eczema are proved. They are able to phagocytize antigens (haptens), to apposite lymphozytes, to proliferate after the challenge by antigens as well as to migrate through the lymph vessels into the regional lymph nodes. 6. At their surface they bear receptors for Fc-IgG and C3 as well as Ia-antigens as immune response genes of the major histocompatibility complex (MHC). 7. With regard to point 5 and 6 they have the same properties as the macrophages. 8. Langerhans cells are damaged and destroyed, respectively, by immune complexes after activation of the complement pathway as well as by killer T-lymphocytes. Thus they are regulated as target cells by humoral and cellular mechanisms. 9. The allergic inflammatory reaction is triggered by mediator substances (of lysosomal origin?) which are liberated by the destruction of the Langerhans cells as well as by lymphokines. The preferently suprabasal occurrence of the Langerhans cells explains the early beginning of the edema and spongiosis as well as their localization in the lower layers of the epidermis. 10. The macrophages-analogous and antigen-presenting functions, which are genetically regulated are essential for the sensitization in allergic contact eczema. The ability to stimulate allogenic T-Lymphocytes in the mixed lymphocyte reaction might be of particular importance for the skin transplantation.
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PMID:[The Langerhans cell--its macrophages-analogous function in the triggering of the allergic contact eczema (author's transl)]. 15 6

The study involves 10 children with primary immunologic deficiency of the humoral and cellular immunity diagnosed on the basis of the clinical symptoms of the disease and immunologic investigation. In 7 children, out of whome three were infants, suffering of recurrent respiratory tract infections, examinations revealed either absence or deficiency of the IgA in the serum and saliva. 2 children presented a classical picture of the Wiskott-Aldrich's syndrome followed by eczema, recurrent infections and trombocytopenia. Having studied the immunologic status in these two children in vitro and in vivo, the authors established deficiency in the humoral and cellular immunity. In an infant aged three months, with diagnosed histiocytosis X after histologic examination of the skin, the authors had examined the function of the T and B cells after which it was concluded that it was a case of rare form of the combined primary immunodeficiency.
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PMID:[Congenital deficiency of humoral and cellular immunity]. 30 54

Thirty-two patients with histiocytosis X were evaluated and treated at Children's Memorial Hospital, Chicago, during the years 1978 to 1984. Twelve patients (38%) had solitary or multifocal bone lesions, three (9%) had bone lesions and diabetes insipidus, and seventeen (53%) had cutaneous and/or multisystem involvement. Age at diagnosis ranged from 2 days to 15 years. Fifteen patients were 2 years of age or younger at the time of diagnosis. Sixteen patients (50%) had skin infiltrates, of whom seven (43%) had cutaneous lesions documented at birth. Cutaneous lesions included vesicopustules, erythematous papules, nodules, eczematous dermatitis, granulomatous ulcerative lesions, petechiae, and hemorrhagic lesions. Xanthomas and nail dystrophy were not observed. The therapeutic regimen chosen was based on extent of involvement and location of infiltrates. Only two of the thirty-two patients died; both had multisystem disease.
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PMID:Histiocytosis X: a seven-year experience at a children's hospital. 387 82

This article discusses many of the more common diagnoses that should be considered when managing a child with nonresponsive excema. Some of these possible diagnoses are infectious and parasitic disorders, psoriasis, heritable disorders, Langerhans cell histiocytosis, and syndromes with altered immunity and eczematous skin changes. This overview of diagnostic and laboratory evaluations of the child with eczema who fails standard therapy is presented as a useful guide in evaluating these patients.
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PMID:Laboratory evaluation of the child with recalcitrant eczema. 814 75

Among the potential sites of involvement by Langerhans cell histiocytosis (LCH), the head and neck region is the most commonly cited. Though principally a pediatric disease, LCH can affect any age group. It can be unifocal (skeletal) or multifocal (skeletal and/or visceral); it appears as though the presence of visceral lesions is more common in the youngest patients, and may be associated in some with a rapidly progressive course resulting in death. Head and neck manifestations may mimic such varied entities as eczema, otitis media, osteomyelitis, and cholesteatoma. Current approaches to therapy are less aggressive than they were in the past, and are particularly intended to monitor for and treat any complicating secondary infections (which may develop in the youngest patients with multifocal disease including visceral involvement). The prognosis is very good for unifocal skeletal system disease, and poor for multifocal disease with involvement of tissues other than bone.
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PMID:Head and neck Langerhans cell histiocytosis. 919 16

We present a very rare congenital immunologic disease, severe combined immunodeficiency syndrome (SCID) in 6-months-old-boy with prolonged mucocutaneous candidiasis, severe anaemia, skin rash similar to the infiltrative eczema of Langerhans cell histiocytosis (LCH) and subcutaneous nodules with histiocytic infiltration. Laboratory findings show profound absence of humoral and cell-mediated immunity. Pathology specimens analysis of subcutaneous nodule revealed numerous S-100 protein and Cd1a negative histiocytes, occupied by BCG intracellular growth. Histopathology and immunohistochemistry confirmed the diagnosis of BCG dissemination. BCG vaccination in infants with SCID can lead to life threatening dissemination, resembling to the infiltrative eczema of LCH and may mislead the clinician.
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PMID:Disseminated BCG infection resembling langerhans cell histiocytosis in an infant with severe combined immunodeficiency: a case report. 1555 21

Langerhans cell histiocytosis (LCH) is a rare hematologic disorder that results from the clonal multiplication and accumulation of immature dendritic Langerhans cells. Its reported incidence rate varies, but is considered to be 2.6-8.9 per million children who are <15 years of age each year. It may affect any system or organ. The present study reported 4 pediatric LCH cases in order to highlight the heterogeneity of the initial presentation, and the pitfalls that may mislead clinicians and delay diagnosis. The clinical features, as well as the pathognomonic imaging, pathology findings and treatment options were presented. LCH may be rare, but it should always be included in the differential diagnosis of persistent eczema, unexplained skin lesions, diabetes insipidus and persistent bone pain, among others. While the debate on pathogenesis and treatment is ongoing, high index of suspicion among pediatricians, pediatric oncologists and other specialists (pathologists, dermatologists, orthopaedic surgeons, general practitioners or family physicians) is essential for early diagnosis, and optimal outcome.
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PMID:The multiple faces of Langerhans cell histiocytosis in childhood: A gentle reminder. 2946 64

Many pediatric skin conditions can be safely monitored with minimal intervention, but certain skin conditions are emergent and require immediate attention and proper assessment of the neonate, infant, or child. We review the following pediatric dermatology emergencies so that clinicians can detect and accurately diagnose these conditions to avoid delayed treatment and considerable morbidity and mortality if missed: staphylococcal scalded skin syndrome (SSSS), impetigo, eczema herpeticum (EH), Langerhans cell histiocytosis (LCH), infantile hemangioma (IH), and IgA vasculitis.
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PMID:Pediatric dermatology emergencies. 3235 37