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- W42777921 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+, α-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 a-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." @default.
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- W42777921 date "1991-05-01" @default.
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- W42777921 title "Congenital Histiocytosis" @default.
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- W42777921 doi "https://doi.org/10.1016/s0344-0338(11)80007-5" @default.
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