A specific ultrastructural marker for disseminated lipogranulomatosis (Faber)

An ultrastructural investigation of two cutaneous lesions in a two-year-old Turkish boy with disseminated lipogranulomatosis (Farber) revealed curvilinear bodies in fibroblasts, histiocytes, and endothelial cells; "elongated membranes" in fibroblasts and endothelial cells; "zebra bodi...

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Bibliographic Details
Published in:Archives of Dermatological Research 1979-01, Vol.266 (2), p.187-196
Main Authors: Schmoeckel, C, Hohlfed, M
Format: Article
Language:English
Subjects:
Child
Child, Preschool
Endothelium - pathology
Fibroblasts - pathology
Granuloma - pathology
Histiocytes - pathology
Humans
Inclusion Bodies - pathology
Inclusion Bodies - ultrastructure
Male
Schwann Cells - pathology
Skin - ultrastructure
Skin Diseases - pathology
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Summary:An ultrastructural investigation of two cutaneous lesions in a two-year-old Turkish boy with disseminated lipogranulomatosis (Farber) revealed curvilinear bodies in fibroblasts, histiocytes, and endothelial cells; "elongated membranes" in fibroblasts and endothelial cells; "zebra bodies" in endothelial cells; and spindle-shaped bodies in Schwann cells. In peripheral lymphocytes only alterations of mitochondria (swelling and ruptured cristae) but no inclusion bodies were found. Curvilinear bodies were numerous and easily identifiable; they appear to be characteristic of Farber's disease, and naming them "Farber bodies" is proposed. The diagnosis of this ceramide storage disease, in which the histological examination is relatively unspecific, can therefore be confirmed ultrastructurally.
ISSN:0340-3696
1432-069X
DOI:10.1007/BF00694628