HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Acute, generalized, malignant course of Langerhans cell histiocytosis in early childhood.
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Occurrence/EpidemiologyThis section has been translated automatically.
The incidence of LCH in childhood is 2.0-5/1,000,000 children. f:m=1.8:1.0
EtiopathogenesisThis section has been translated automatically.
- Unknown; family cases with autosomal recessive inheritance have been described.
- Pathogenetic: Proliferation of dendritic cells (Langerhans cells) with expression of CD1 antigen, S-100 protein and neuron-specific endase (NSE).
ManifestationThis section has been translated automatically.
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Clinical featuresThis section has been translated automatically.
- Integument: Eruption of disseminated small, flat, yellow-brownish papules with scaly to crusty surface. Tendency to necrotic disintegration and petechial haemorrhages, haemorrhagic eczematoid, sometimes ulcerated lesions, especially capillitium, intertriginous spaces, joint folds affected; aphthous lesions of the oral mucosa; thrombocytopenic purpura.
- Extracutaneous manifestation: Destructive bone foci (extremities, ribs, skull, iliac crest), scarring. Obligatory swelling of lymph nodes, hepatosplenomegaly, fever, increasing anemia. Optional pulmonary involvement (cough, dyspnoea, radiological miliary speckling), osteolysis.
LaboratoryThis section has been translated automatically.
Leukocytosis or leukopenia, thrombopenia, hypochromic anemia.
HistologyThis section has been translated automatically.
- Dense subepidermal infiltrate of large, atypical histiocytoid cells with kidney-shaped nuclei as well as lymphocytes and possibly eosinophil granulocytes with distinct epidermotropism. Immunohistochemically, the cells react with S100, CD1a, and Langerin-CD207.
- Electron microscopy: detection of Langerhans cell granules (tennis racket-like Birbeck granules)
Differential diagnosisThis section has been translated automatically.
External therapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Abbot AF, Denenholz EJ (1936) Letterer-Siwe disease. On J Dis Child 51: 499-522
- Feyrter F (1955) On the relationships between Abt-Letterer-Siweschen disease, eosinophilic bone granuloma (eosinophilic granulomatosis) and Hand-Schüller-Christian disease. Medical, Stuttgart: 1019-1025
- Letterer E (1924) Aleukemic reticulosis. A contribution to the proliferative diseases of the reticuloendothelial apparatus. Frankf Zschr Path 30: 377
- Seward JL et al (2004) Generalized eruptive histiocytosis. J Am Acad Dermatol 50: 116-120
- Siwe SA (1933) Reticuloendotheliosis, a new clinical picture among hepatosplenomegaly Zschr Pediatrics 55: 212
- Vade A, Hayani A, Pierce KL (1993) Congenital histiocytosis X. Pediatric radiol 23: 181-182
- Wolff HH, Janka GE (1978) Abt-Letterer-Siwe disease. For diagnosis and therapy. Mschr pediatrics 126: 425-430
- Willman CL et al (1994) Langerhans-cell histiocytosis (histiocytosis X)--a clonal proliferative disease. N Engl J Med 331: 154-160
Incoming links (9)Granuloma eosinophiles; Histiocytosis x, acute disseminated juvenile form; Letterer-siwe disease; Letterer-siwe, m.; Pustulose sterile eosinophils; Reticuloendotheliosis, acute; Reticulohistiocytosis of the skin with benign course; Reticulosis, aleukemic; Tuberculosis cutis miliaris disseminata;
Outgoing links (12)Antibiotics; Cytostatics (overview); Dyskeratosis follicularis; Glucocorticosteroids; Glucorticosteroids topical; Haemophagocytic lymphohistiocytosis, familial; Langerhans cell histiocytosis (overview); Leukemias of the skin; Papel; Scale; ... Show all
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