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Case report: Erdheim-Chester disease: Polyostotic sclerosing histiocytosis

1986; Elsevier BV; Volume: 37; Issue: 1 Linguagem: Inglês

10.1016/s0009-9260(86)80184-2

1365-229X

Sharon Evans, Frank Williams,

Genetic and rare skin diseases.

Erdheim-Chester disease is characterised by diffuse metaphyseal and diaphyseal sclerosis of long tubular bones of the appendicular skeleton. In addition, there is an inconstant tendency to visceral involvement by lipogranulomatous infiltration. Histologically, this condition has marked similarities to Hand-Schuller-Christian disease. A further case is presented which supports the view that Erdheim-Chester disease is part of the spectrum of histocytoses and not a distinct pathological entity. Polyostotic sclerosing histiocytosis would be an appropriate alternative label for this condition. Erdheim-Chester disease is characterised by diffuse metaphyseal and diaphyseal sclerosis of long tubular bones of the appendicular skeleton. In addition, there is an inconstant tendency to visceral involvement by lipogranulomatous infiltration. Histologically, this condition has marked similarities to Hand-Schuller-Christian disease. A further case is presented which supports the view that Erdheim-Chester disease is part of the spectrum of histocytoses and not a distinct pathological entity. Polyostotic sclerosing histiocytosis would be an appropriate alternative label for this condition.