Retinal manifestations of xanthoma disseminatum (Non-Langerhans cell histiocytosis)

Details

Serval ID
serval:BIB_3090865DA6E0
Type
Inproceedings: an article in a conference proceedings.
Publication sub-type
Abstract (Abstract): shot summary in a article that contain essentials elements presented during a scientific conference, lecture or from a poster.
Collection
Publications
Institution
Title
Retinal manifestations of xanthoma disseminatum (Non-Langerhans cell histiocytosis)
Title of the conference
Ophta 2010
Author(s)
Konstantinidis  Lazaros
Organization
103e Congrès annuel de la Société Suisse d’Ophtalmologie
Address
Montreux Music & Convention Center MMCC
Grand-Rue 95,
P.O. Box 1151,
CH-1820 Montreux
Publication state
Published
Issued date
18/09/2010
Pages
S80
Language
english
Abstract
Xanthoma disseminatum (XD) is a rare normolipemic histiocytic disorder of non-Langerhans cell origin. Apart from eye- lids involvement, only sporadic reports of ocular involvement have been described. As far as we are aware this is the first report of retinal manifestations related to XD. History and Signs A 57-year-old woman with known history of XD which occurred 25 years previ- ously (Montgomery syndrome), presented with blurred vision in her RE. She had inactive skin and pulmonary lesions. Visual acuity (VA) at presentation was 1.0. Multiple intraretinal yellow deposits were present with pigment epithelium alterations superior to the macula. Fluorescein angiography revealed ill-defined hyperfluo- rescence while the ultrasound revealed a thickening of the sclera. Fifteen months later the patient VA was 0.5 associated with shal- low subfoveal fluid accumulation and lipid infiltration. Therapy and Outcome Oral steroid treatment led to clinical, anatomical and functional improvement after a period of 6 months. VA improved to 1.0 at the last follow-up. Conclusions The present report illustrates a case that may represent an active chorioretinal disseminated XD. Oral steroids treatment may be an efficacious treatment.
Create date
06/09/2019 12:22
Last modification date
07/09/2019 5:26
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