[Self-healing childhood histiocytosis X (Illig-Fanconi disease). Comments on ultrastructural aspects and etiopathogenic classification of histiocytosis]. 1982

J Ferrando, and T Estrach, and J A Bombi, and S Bassas, and E Navarra, and A Simon

Two cases of cutaneous self-healing histiocytosis X in a 6 and a 16 months-old children, are reported. Clinically, the lesions were characterized by few, small, translucent and confluent papules, sometimes purpuric. The scalp lesions were seborrheic eczema-like; the elements on the groins were erosive. Systemic examinations, laboratory data and general development were completely normal. The histological pattern, in both cases, were identical of that in histiocytosis X (atypic mononuclear cells with glassy eosinophilic cytoplasm and excentric kidney-shaped nucleus). Electron microscopy of the second case showed a proliferation of histiocytes containing Langerhans bodies. Ten per cent of these cells showed multivesicular, myelin and dense laminar bodies. The latest had been specially described in other selfhealing histiocytic syndromes: congenital reticulohistiocytosis (Hashimoto-Pritzker) and generalized eruptive histiocytoma. Since the clinical behaviour and prognosis of the histiocytic proliferations can not be assumed on clinical and histological data, an attempt, of classification and understanding of these processes is presented. The possibility that histiocytosis represent a proliferation of the mononuclear-phagocytic system at different levels of cellular maturation, is considered. So it would be possible to consider: 1) Acute malignant processes: xantholeukaemia, malignant histiocytosis syndromes (histiocytic medullary reticulosis . . .), and Letterer-Siwe disease; 2) Chronic and severe processus: xanthoma disseminatum, necrobiotic xanthogranuloma, disseminated plane xanthoma, multicentric reticulohistiocytosis, localized reticulohistiocytoma of Crosti and Hand-Schüller-Christian disease; and 3) Benign processus, some of them being involutive: juvenile xanthogranuloma, regressing atypical histiocytosis, eosinophilic granuloma of the bone, Illig-Fanconi disease, Hashimoto-Pritzker disease, benign cephalic histiocytosis and generalized eruptive histiocytoma. These groups include classic X-histiocytosis, self-healing X-histiocytosis, non-X malignant histiocytosis and non-X benign or auto involutive-histiocytosis (some with intracellular lipid storage: xanthohistiocytosis) (see table II). The ultrastructural cytoplasmic markers of histiocytosis (table I) are consistent features in the accuracy of diagnosis of these conditions.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007801 Langerhans Cells Recirculating, dendritic, antigen-presenting cells containing characteristic racket-shaped granules (Birbeck granules). They are found principally in the stratum spinosum of the EPIDERMIS and are rich in Class II MAJOR HISTOCOMPATIBILITY COMPLEX molecules. Langerhans cells were the first dendritic cell to be described and have been a model of study for other dendritic cells (DCs), especially other migrating DCs such as dermal DCs and INTERSTITIAL DENDRITIC CELLS. Langerhans Cell,Dendritic Cells, Dermal,Dendritic Cells, Epidermal,Dendritic Cells, Skin,Dermal Dendritic Cells,Epidermal Dendritic Cells,Skin Dendritic Cells,Cell, Dermal Dendritic,Cell, Epidermal Dendritic,Cell, Langerhans,Cell, Skin Dendritic,Cells, Dermal Dendritic,Cells, Epidermal Dendritic,Cells, Langerhans,Cells, Skin Dendritic,Dendritic Cell, Dermal,Dendritic Cell, Epidermal,Dendritic Cell, Skin,Dermal Dendritic Cell,Epidermal Dendritic Cell,Skin Dendritic Cell
D008297 Male Males
D008854 Microscopy, Electron Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen. Electron Microscopy
D005260 Female Females
D006646 Histiocytosis, Langerhans-Cell A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder. Histiocytosis X,Langerhans-Cell Granulomatosis,Langerhans-Cell Histiocytosis,Hand-Schueller-Christian Disease,Hand-Schueller-Christian Syndrome,Hand-Schüller-Christian Disease,Hand-Schüller-Christian Syndrome,Hashimoto-Pritzger Disease,Histiocytosis, Generalized,Histiocytosis-X,Langerhans Cell Granulomatosis,Langerhans Cell Granulomatosis, Pulmonary,Langerhans Cell Histiocytosis,Letterer-Siwe Disease,Non-Lipid Reticuloendotheliosis,Pulmonary Histiocytosis X,Pulmonary Langerhans Cell Granulomatosis,Schueller-Christian Disease,Systemic Aleukemic Reticuloendotheliosis,Type 2 Histiocytosis,Aleukemic Reticuloendothelioses, Systemic,Aleukemic Reticuloendotheliosis, Systemic,Cell Granulomatoses, Langerhans,Cell Granulomatosis, Langerhans,Cell Histiocytoses, Langerhans,Cell Histiocytosis, Langerhans,Disease, Hand-Schueller-Christian,Disease, Hand-Schüller-Christian,Disease, Hashimoto-Pritzger,Disease, Letterer-Siwe,Disease, Schueller-Christian,Diseases, Hashimoto-Pritzger,Generalized Histiocytoses,Generalized Histiocytosis,Granulomatoses, Langerhans Cell,Granulomatosis, Langerhans Cell,Granulomatosis, Langerhans-Cell,Hand Schueller Christian Disease,Hand Schueller Christian Syndrome,Hand Schüller Christian Disease,Hand Schüller Christian Syndrome,Hashimoto Pritzger Disease,Hashimoto-Pritzger Diseases,Histiocytoses, Generalized,Histiocytoses, Langerhans Cell,Histiocytoses, Type 2,Histiocytosis X, Pulmonary,Histiocytosis, Langerhans Cell,Histiocytosis, Type 2,Langerhans Cell Granulomatoses,Langerhans Cell Histiocytoses,Letterer Siwe Disease,Non Lipid Reticuloendotheliosis,Non-Lipid Reticuloendothelioses,Reticuloendothelioses, Non-Lipid,Reticuloendothelioses, Systemic Aleukemic,Reticuloendotheliosis, Non-Lipid,Reticuloendotheliosis, Systemic Aleukemic,Schueller Christian Disease,Syndrome, Hand-Schueller-Christian,Syndrome, Hand-Schüller-Christian,Systemic Aleukemic Reticuloendothelioses,Type 2 Histiocytoses
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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