title: Congenital histiocytosis. A heterogeneous group of diseases, one presenting as so-called congenital self-healing histiocytosis.
abstract: Three cases of congenital histiocytic disorders--generalized Langerhans cell histiocytosis, generalized juvenile xanthogranuloma and so-called congenital self-healing histiocytosis are compared using histiochemical, immunohistochemical and ultrastructural methods. The results showed a typical morphological pattern of Langerhans cell histiocytosis (S 100+, CD 1+, alpha-mannosidase +) with an unusual self-healing cutaneous phenomenon. The congenital self-healing histiocytosis showed a non-Langerhans cell immunophenotype (CD 14+, CD 1-, S 100-) and morphological appearance resembling the evolutive "early" stage of juvenile xanthogranuloma. A diffuse cellular positivity of alpha-mannosidase in juvenile xanthogranuloma and congenital self-healing histiocytosis differed from a typical perinuclear globular positivity of this enzyme in Langerhans cell histiocytosis. It is concluded that congenital self-healing histiocytosis may in some cases be of non-Langerhans cell type and under this term a clinically characteristic syndrome of histiocytic proliferation of Langerhans cells or tissue histiocytes may be included.
authors.affiliation: Department of Pathology, Faculty of Paediatric Medicine, Charles' University, Prague, Czechoslovakia.
authors.forename: R
authors.initials: R
authors.lastname: Kodet
authors.forename: M
authors.initials: M
authors.lastname: Elleder
authors.forename: C
authors.initials: C
authors.lastname: De Wolf-Peeters
authors.forename: H
authors.initials: H
authors.lastname: Mottl
mesh_terms: D015415:Biomarkers; D006644:Histiocytes / Q000201:enzymology / Q000648:ultrastructure; D015614:Histiocytosis / Q000151:congenital* / Q000201:enzymology / Q000473:pathology*; D006646:Histiocytosis, Langerhans-Cell / Q000151:congenital / Q000473:pathology; D006651:Histocytochemistry; D006801:Humans; D007124:Immunoenzyme Techniques; D007231:Infant, Newborn; D008297:Male; D014972:Xanthogranuloma, Juvenile / Q000151:congenital / Q000473:pathology
chemical_list: D015415:Biomarkers