Ocular Findings In X-Linked Ichthyosis - A Case Report .

X-linked ichthyosis is a rare skin disease belonging to the Mendelian Disorders of Cornification (MeDOC), characterized by generalized hyperkeratosis and skin scaling. Ocular damage during X-linked ichthyosis is very common, mainly in the anterior segment such as: Ectropion, blepharitis, conjunctivitis, dry eye, Superficial punctate keratitis, corneal dystrophies

We report the case of a child aged of 06 years , followed in dermatology for hereditary ichthyosis linked to the X; who presents ocular symptoms such as photophobia, blepharospasm, foreign body sensation , recurrent painful red eye. Knowing that the child's parents report an increase in signs every winter 

The general clinical examination of the child reveals an appearance of generalized hyperkeratosis with scaly skin especially on the trunk, upper and lower limbs.
Inspection of the ocular apparatus reveals a
photophobic child, with significant blepharospasm, and watery red eyes. The examination of ocular motility is unremarkable as is corneal sensitivity. 
The child's cycloplegic refraction is as follows:
OD: Sphere +2.25. Cylinder + 1.00 at 80°
OG: Sphere + 2.00. Cylinder + 1.50 at 90°
Distant visual acuity with correction: - Right Eye: 04/10. - Left Eye: 03/10.
Examination of the anterior segment with the slit lamp of the eyes reveals a slightly asymmetrical bilateral picture: Wet, stuck eyelashes, telangiectasias and diffuse hyperemia of the free edge with keratinized plugs of the meibomian gland meatuses, Conjunctival hyperemia with a few follicles especially in nasal. The fluorescein examination found a break-up time reduced to 06 seconds; with superficial epithelial lesions concentrated in the interpalpebral area or lower. The rest of the ophthalmological clinical examination is normal . 
Ocular damage during X-linked ichthyosis shows a significant prevalence and severity of dry eye, blepharitis and superficial punctate keratitis . The aim of treating eye damage in the replacement of the ocular surface with artificial tears. These disorders do not respond to corticosteroids .

X-linked ichthyosis is a rare hereditary skin disease characterized by generalized hyperkeratosis which can involve the eyelids and therefore affecting the ocular surface leading to ocular damage of varying complexity. Prevention is mainly based on genetic counseling