A specific ultrastructural marker for disseminated lipogranulomatosis (Faber).

Abstract:

: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.

journal_name

Arch Dermatol Res

authors

Schmoeckel C,Hohlfed M

doi

10.1007/BF00694628

subject

Has Abstract

pub_date

1979-10-01 00:00:00

pages

187-96

issue

2

eissn

0340-3696

issn

1432-069X

journal_volume

266

pub_type

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