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 Table of Contents    
CLINICAL IMAGE
Year : 2018  |  Volume : 11  |  Issue : 1  |  Page : 88-89  

Ophthalmologic manifestations of pseudoxanthoma elasticum


Department of Ophthalmology, La Marche Verte Hospital, Missour, Morocco

Date of Web Publication5-Mar-2018

Correspondence Address:
Khrifi Zineb
B.P 2193, V.N, Fez
Morocco
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ojo.OJO_123_2016

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   Abstract 


Pseudoxanthoma elasticum is a hereditary disorder that affects primarily the elastic tissues in the skin, the eyes and the blood vessels.

Keywords: Angioid, manifestations, ocular, pseudoxanthoma elasticum, streaks


How to cite this article:
Zineb K. Ophthalmologic manifestations of pseudoxanthoma elasticum. Oman J Ophthalmol 2018;11:88-9

How to cite this URL:
Zineb K. Ophthalmologic manifestations of pseudoxanthoma elasticum. Oman J Ophthalmol [serial online] 2018 [cited 2020 Aug 15];11:88-9. Available from: http://www.ojoonline.org/text.asp?2018/11/1/88/226332



Pseudoxanthoma elasticum (PXE) is a hereditary disorder that affects primarily the elasctic tissues in the skin [Figure 1], the eyes, and the blood vessels.[1]

In the eye, the outer layer of the retina, Bruch's membrane, contains elastic fibers. Loss of elasticity and enhanced calcification lead to cracks in Bruch's membrane, which resemble retinal vessels and thus are called angioid streaks of the retina, leading usually after age 40 years to hemorrhages and retinal scarring with as a result in most patients macular degeneration and vision loss.[2],[3]
Figure 1: skin changes (yellow papules) in a patient with pseudoxanthoma elasticum

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Ocular signs eventually develop in most patients with PXE. The usual sequence of developing eye abnormalities is peau d'orange [Figure 2] and [Figure 3] or mottled hyperpigmentation of the retina, angioid streaks [Figure 4] and [Figure 5]. Other retinal lesions: pattern dystrophy, drusen of the optic nerve, peripheral “comet tails” lesions, subretinal fibrosis, and autofluorescent perimacular white spots. The risk of neovascular complications increased with age.[4],[5]
Figure 2: Fundus photograph of the right eye with the “peau d'orange” appearance. Note the barely visible angioid streaks

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Figure 3: Fundus photograph of the left eye with the “peau d'orange” appearance and barely visible angioid streaks

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Figure 4: Fluorescein angiography of the right eye depicting the angioid streaks in great detail

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Figure 5: Fluorescein angiography of the left eye showing the angioid streaks in great detail

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We report the case of 38-year-old female presenting yellow soft papules affecting the neck and axillar area. The fundus examination reveals peau d'orange aspect in both eyes and angioid streaks.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Georgalas I, Tservakis I, Papaconstaninou D, Kardara M, Koutsandrea C, Ladas I. Pseudoxanthoma elasticum, ocular manifestations, complications and treatment. Clin Exp Optom 2011;94:169-80.  Back to cited text no. 1
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2.
Andonaba JB, Barro-Traoré F, Korsaga S, Diallo B, Diallo JW, Traoré A. Generalized elastic and evolving pseudoxanthoma in a black. Pan Afr Med J 2013;16:132.  Back to cited text no. 2
    
3.
Bercovitch L, Terry P. Pseudoxanthoma elasticum 2004. J Am Acad Dermatol 2004;51 1 Suppl: S13-4.  Back to cited text no. 3
    
4.
Gliem M, Müller PL, Birtel J, Hendig D, Holz FG, Charbel Issa P. Frequency, phenotypic characteristics and progression of atrophy associated with a diseased bruch's membrane in pseudoxanthoma elasticum. Invest Ophthalmol Vis Sci 2016;57:3323-30.  Back to cited text no. 4
    
5.
Orssaud C, Roche O, Dufier JL, Germain DP. Visual impairment in pseudoxanthoma elasticum: A survey of 40 patients. Ophthalmic Genet 2015;36:327-32.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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