HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Generic term for a group of differently progressing systemic diseases, different dignities, with proliferation of dendritic cells of the Langerhans cell type and ontogenetically related cell variants. The proliferating cell, originally designated "X", has now been identified as a Langerhans cell, which shows Langerhans cell granules (Birbeck granules) under the electron microscope and stains itself immunohistologically with Langerhans cell markers ( CD1a), so that the term histiocytosis X (Lichtenstein) is no longer adequate.
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ClassificationThis section has been translated automatically.
Langerhans cell histiocytoses are divided into: Classical Langerhans cell histiocytoses (CD1a+, Birbeck granula+):
- Acute multifocal and multisystemic (disseminated) Langerhans cell histiocytosis(Abt-Letterer-Siwe disease): acute and severe course, in infants and young children.
- Chronic multifocal Langerhans cell histiocytosis(Hand-Schüller-Christian disease)-including adult Langerhans cell histiocytosis-: chronic, adult onset, chronic course, often xanthomatous skin lesions.
- Unifocal Langerhans cell histiocytosis(eosinophilic granuloma of bone; rarely occurring pulmonary - pulmonary Langerhans cell histiocytosis): Chronic, granulomatous, in late adolescence and adults.
Precursor Langerhans cell histiocytoses (CD1a+; Birbeck granula+/-):
- Congenital self-healing reticulohistiocytosis Hashimoto-Pritzker (CD1a+; Birbeck granules +/-).
- Histiocytosis of indeterminate cells (CD1a+; Birbeck granula+).
Instead of the previously listed classification according to entities (probably these are not entities but only different manifestations and courses of a single underlying disease), a stratification for the classical Langerhans cell histiocytoses has proven useful, especially for therapy studies: "single-system-disease":
- monoorganic affection of the skin. Unilocular skeletal infestation, infestation of a solitary lymph node .
- Disseminated (organ) involvement: multilocular skeletal involvement; involvement of multiple lymph nodes.
- Involvement of multiple organ systems
- High risk: patient > 2 years; no involvement of hematopoietic system, liver, lung, or spleen.
- Patient < 2 years, or patient > 2 years with involvement of the hematopoietic system, liver, lung, or spleen.
Occurrence/EpidemiologyThis section has been translated automatically.
Incidence is 0.4 (0.2-1.0)/100,000 for children < 15 years of age; less common is adult onset.
EtiopathogenesisThis section has been translated automatically.
ManifestationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
The clinical picture is very variable and is characterized by the different organ manifestations (see under the different variants). In children in particular, the spectrum ranges from asymptomatic symptoms to discrete localised complaints to generalised symptoms such as weight loss, failure to thrive, restlessness and fever (see below Abt-Letterer-Siwe disease). The most frequent localization is the skeleton with about 80% (see below Eosinophilic granuloma of the bone - 34% skull -), followed by the skin manifestation (60%).
- Skin involvement varies according to the acuity of the disease. Disseminated, small, flat, yellow-brownish papules with scaly to crusty surfaces are found. There is a tendency to necrotic disintegration and petechial haemorrhages, haemorrhagic-eczematoid ulcers and, in places, smaller ulcers. Especially affected in children are capillitium, intertriginous spaces, joint folds; furthermore, aphthous lesions of the oral mucosa; HV also hemorrhagic or xanthomatous.
- In disseminated forms, one sees various combinations of rash, hepatosplenomegaly, generalized lymph node swelling, anemia or pancytopenia, pulmonary involvement, or bone involvement. Dysfunction of the liver (increased transaminases, hypoproteinemia, hypoalbuminemia, hyperbilirubinemia), lungs (dyspnea, pneumothorax), and hematopoiesis (cytopenia with or without bone marrow involvement).
- General symptoms in osseous manifestations are local swelling, pain or limitation of movement. Depending on further organ involvement, other symptoms such as otitis media, otorrhea, loosening or loss of teeth, exophthalmos (see Hand-Schüller-Christian disease) or pathological fractures may be detectable.
- CNS involvement, often with a chronic course, may occur . Diabetes insipidus, as a sign of hypothalamic-pituitary CNS involvement, can often occur years before or after disease manifestations in other regions.
HistologyThis section has been translated automatically.
TherapyThis section has been translated automatically.
- Chemotherapy: Various study protocols are described. Involved cytostatics: 6-mercaptopurine; vinblastine; implementation by pediatric oncologists.
- Salvage therapy: Immunosuppressive therapy (induction: ATG, prednisolone; maintenance: Ciclosporin A).
- Allogeneic bone marrow transplantation in advanced stages.
- Since about 50% of Langerhans cell histiocytoses have a BRAF V600E mutation (this also applies to systemic non-Langerhans cell histiocytoses ), very good therapeutic effects can be achieved with the BRAF inhibitor vemurafinib.
Progression/forecastThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Birbeck MS et al (1961) An electron microscope study of basal melanocytes and high-level clear cells (Langerhans cells) in vitiligo. J Invest Derm 37: 51-64
- Goerdt S (1998) Histiocytic tumours. In: Garbe C, Rassner G Dermatology, guidelines and quality assurance for diagnostics and therapy. Springer Verlag, Berlin Heidelberg New York S 372-378
- Langerhans P (1868) On the nerves of human skin. Virchows Arch Catholic Anat 44: 325
- Leonidas JC et al (2003) Langerhans' cell histiocytosis. Lancet 361: 1293-1295
- Lichtenstein L (1953) Histiocytosis X. Integration of eosinophilic granuloma of bone, Letterer-Siwe Disease, and Schüller-Christian Disease as related manifestations of a single nosologic entity. Arch Catholic 56: 84-102
- Misery L (2003) Malignant Langerhans cell tumor: a case with a favorable outcome associated with the absence of blood dendritic cell proliferation. J Am Acad Dermatol 49: 527-529
- Petersen BL et al (2003) High expression of markers of apoptosis in Langerhans cell histiocytosis. Histopathology 42: 186-193
- Ruzicka T, Evers J (2003) Clinical course and therapy of Langerhans cell histiocytosis in children and adults. dermatologist 54: 148-155
- The writing Group of the Histiocyte Society (1987) Histiocytosis syndromes in children. Lancet I: 208-209
Incoming links (28)Abt-letterer-siwe disease; Christian disease of the hand pupil; Chronic non-bacterial osteomyelitis; Dental diseases, skin changes; Diaper dermatitis; Eosinophilia skin changes; Ergotism, anocutaneous gangraenous; Granuloma eosinophiles; Haemophagocytic lymphohistiocytosis, familial; Histiocytosis benign cephalic; ... Show all
Outgoing links (10)Abt-letterer-siwe disease; Braf inhibitors; Cd classification; Christian disease of the hand pupil; Ciclosporin a; Granuloma eosinophiles; Histiocytoses non-langerhans cell histiocytoses; Langerhans cell; Pulmonary langerhans cell histiocytosis; Reticulohistiocytosis of the skin with benign course;
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