Unilateral superior pellucid marginal degeneration in a case with ichthyosis

Unilateral superior pellucid marginal degeneration in a case with ichthyosis

Contact Lens & Anterior Eye 34 (2011) 45–48 Contents lists available at ScienceDirect Contact Lens & Anterior Eye journal homepage: www.elsevier.com...

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Contact Lens & Anterior Eye 34 (2011) 45–48

Contents lists available at ScienceDirect

Contact Lens & Anterior Eye journal homepage: www.elsevier.com/locate/clae

Case report

Unilateral superior pellucid marginal degeneration in a case with ichthyosis Huseyin Dundar, Necip Kara ∗ , Vedat Kaya, Ercument Bozkurt, Ahmet Taylan Yazici, Pelin Kaynak Hekimhan Beyoglu Eye Research and Education Hospital, Istanbul, Turkey

a r t i c l e

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Keywords: Corneal topography Ichthyosis vulgaris Superior pellucid marginal degeneration

a b s t r a c t A 47-year-old man with ichthyosis vulgaris presented to our hospital complaining of reduced visual acuity and ocular discomfort in the left eye. Slit-lamp biomicroscopy revealed a thinning about 2 mm from the superior limbus and superficial punctate corneal lesions in the left eyes. Corneal topography was ‘butterfly-like’ in an area of increased elevation in the left eye. Although ichthyosis vulgaris and unilateral superior pellucid marginal degeneration are both uncommon conditions, this is first report about these two conditions in studied together. Crown Copyright © 2010 Published by Elsevier Ltd on behalf of British Contact Lens Association. All rights reserved.

1. Introduction Ichthyosis is a condition characterized by excessive cutaneous dryness and scales. It has four types including ichthyosis vulgaris, sex-linked recessive, lamellar ichthyosis, and epidermolytic hyperkeratosis [1]. Ichthyosis vulgaris is the most common form of ichthyosis and has autosomal dominant inheritance. Different manifestations on some ocular and adnexal structures may accompany ichthyosis. Here we report a unilateral superior pellucid marginal degeneration in a case with ichthyosis vulgaris. 2. Case report A 47-year-old man presented to our hospital complaining of gradual decrease in visual acuity for one year and ocular discomfort in his left eye. The patient gave a history of bilateral chronic eye rubbing which was more severe in the left eye. He used topical medication for this complaint. Also, his medical history was remarkable for the diagnosis of ichthyosis vulgaris since childhood. His sister and first cousin had also been diagnosed with ichthyosis vulgaris. However, they did not come to ophthalmological examination. We asked the patient whether his relatives wore spectacles and had any ocular complaints. He stated he was not sure whether his cousin had any ocular complaints. He reported his sister being a spectacle wearer. His best corrected visual acuity was 20/25 with

∗ Corresponding author at: Kartaltepe Mh. Akın Sk. Akın Apt. No:8/14 Bakırköy, Istanbul, Turkey. Tel.: +90 05058666830. E-mail address: [email protected] (N. Kara).

−1.50 × 20◦ in the right eye (OD) and 20/200 with −6.50–11.0 × 70◦ in the left eye (OS). Ocular examination disclosed hyperkeratotic lesions on the eyelid and scales on the lashes in the right and left eye (Fig. 1). Slit-lamp biomicroscopy revealed a thinning of about 2 mm from the limbus extending from 3 to 9 o’clock positions in the superior half of limbus and superficial punctate corneal lesions in the left eye (Fig. 2). Slit-lamp examination of the right cornea revealed normal features without any thinning. The intraocular pressure measured with pneumotonometer was 12 mm Hg in the right eye and 14 mm Hg in the left eye. Dilated fundus examination revealed no abnormalities in either eye. Orbscan findings showed an area of increased elevation corresponding to the area of ectasia in the left eye (Fig. 3). The topography of the right eye was normal (Fig. 4). A rigid gas permeable contact lens was fitted to left eye achieving a VA of 20/100. However, due to the ocular discomfort, the patient discontinued the contact lens treatment. The patient also declined scleral lens treatment and any surgical procedures. 3. Discussion Pellucid marginal corneal degeneration, which is a progressive noninflammatory ectatic disorder, is usually located in the inferior cornea extending from the 4 o’clock position to the 8 o’clock position and 1.0–2.0 mm from the limbus. Although the exact incidence and prevalence are unknown, unilateral superior PMCD is a very rare corneal ectatic disorder. No gender predilection has been found in most of published series. However, some articles suggested that more males are affected [2–4]. Patients usually present with reduced visual acuity resulting from irregular astigmatism. The typical corneal topographic findings of PMCD are a classical

1367-0484/$ – see front matter. Crown Copyright © 2010 Published by Elsevier Ltd on behalf of British Contact Lens Association. All rights reserved. doi:10.1016/j.clae.2010.09.001

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Fig. 1. Photo of the left eyelid shows the crust on the eyelid and scales on the eyelashes.

Fig. 2. Slit view of the patient with superior pellucid marginal corneal degeneration in the left eye.

“butterfly” pattern, reduced power in the vertical meridian and superior loop cylinder [2,5,6]. PMCD may be differentiated from other corneal ectatic disorder such as keratoconus with a peripheral location of the thinning, the older age group (4–5 decade), lack of a conical shape, lack of inflamatuar sign, and classic butterfly pattern on corneal topography [7,8]. Although PCMD has classically been described as inferior, atypical locations have also been reported. Rao et al. reported topographic features of PMCD with atypical locations, including one case of nasal PMCD and three cases of superior PMCD [9]. Sridhar et al. reported 15 eyes with superior PMCD; the extent of thinning was commonly seen between the 10 and 2 o’clock positions [10]. Although PMCD is a bilateral condition, unilateral involvement has also been reported [11,12]. However, there are a few reports on a unilateral superior PMCD [10,13]. In our patient, a superior PMCD was seen between the 3 o’clock position and the 9 o’clock position in the left eye. Although various ocular manifestations have been reported in ichthyosis, there was no report on superior PMCD with ichthyosis.

Haritoglou et al. reported the corneal manifestations in two brothers with X-linked ichthyosis. He found flour-like and diffusely distributed opacities of the posterior corneal stroma and superficial, small, granular opacities [14]. Piccirillo et al. reported on ophthalmological examinations in 32 male patients affected by ichthyosis; they have found corneal opacities in only five patients [15]. In our case, the biomicroscopic examination revealed superior corneal thinning and superficial punctuate corneal lesions leading to ocular discomfort. Moreover, ectropion, spontaneous corneal perforation, posterior embryotoxon, and unilateral megalocornea have been reported to be involved in ichthyosis [16–18]. Furthermore, there has been a report of association of PMCD with scleroderma [19]. An association was described between ichthyosis vulgaris and atopic conditions such as atopic dermatitis, allergic rhinoconjunctivitis and asthma [20–22]. Also, atopic ocular conditions like atopic keratoconjunctivitis may be a contributory factor in the development of corneal ectatic disorders such as keratoconus and PMCD [23–25]. Jain et al. reported a case of atopic dermatitis and atopic

Fig. 3. Corneal topography of left eye discloses the superior PMCD between the 3 o’clock and 9 o’clock positions.

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Fig. 4. Normal topographic appearance of the right eye.

keratoconjunctivitis with associated PMCD [26]. Various studies reported chronic eye rubbing as a major risk factor for development of corneal ectatic disorders [26–28]. Moreover, eye rubbing was found to be a feature of number of conditions linked to keratoconus such as Down syndrome, mental retardation and Leber’s congenital amarosis [29,30]. Chronic eye rubbing as a common factor in above mentioned conditions may be accused of the development of PMCD in the present case. In the early stages of PMCD, spectacles and contact lenses are the usual treatment modalities for the improvement of visual acuity. If patients cannot be rehabilitated with spectacle correction or contact lenses, a surgical procedure such as lamellar keratoplasty or penetrating keratoplasty can be used [6]. The surgical interventions should be delayed as far as possible, due to intraoperative complications and poor long-term results because of thinning adjacent to the limbus [2,19]. Contact lens fitting is difficult in cases with pellucid marginal corneal degeneration because of central steepening [5]. The anterior corneal surface in cases with superior PMCD may cause insufficient correlation between anterior corneal surface and posterior surface of contact lenses [4]. In these cases, a scleral lens may be tried [2]. A contact lens treatment was applied to our patient. Because of the ocular discomfort, the contact lens treatment was discontinued, while scleral lens and surgical treatment was declined by the patient. Ichthyosis vulgaris is a cutaneous disorder and it is associated with atopic conditions that may be a contributory factor in the development of PMCD. Hence, an association between ichthyosis vulgaris and PMCD may not be surprising. PMCD should be considered in the differential diagnosis of superior corneal ectatic disorders such as superior keratoconus and Terrien’s Marginal Degeneration especially corneas affected by cutaneous disorders such as ichthyosis. Competing interest None. Funding/support None.

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