The Ophthalmology Unit,  Universiti Malaysia Sarawak (UNIMAS), Kuching, Sarawak.
The Ophthalmology Department, Sarawak General Hospital, Kuching, Sarawak, East Malaysia.
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aCase 6a

by Dr. Zamri and Dr.Chua

This 21-year-old man was referred by the dermatologist because of ocular irritation. He had this skin condition through out his body since birth. There was no family history of note. His development was otherwise normal. His vision was 6/6 in both eyes and there were no changes in the anterior segment.

 

Facial appearance

　

On lid closure

　

a. What do the pictures show?

  • Bilateral upper and lower lids ectropion.

  • Right conjunctival exposure on lid closure.

  • Dry skin with excessive scaling.
    　

b. What is the most likely diagnosis?

　

Lamellar ichthyosis.

　

The word ichthyoses is derived from the Greek word ichthys, meaning fish because of the scales produced by this skin condition. Lamellar ichthyosis is an autosomal recessive disorder. Scales are seen throughout the body and are easily removed.  The sales appear large, flat, polygonal and dark as in this case and usually come off in sheets. The skin shows cellular hyperplasia with an increased rate of cell formation in the dermis and cell accumulation in the epidermis. The skin tightness and dryness are caused by an increased transepidermal loss of water causing dehydration of the stratum corneum. The skin condition usually improves with age.1

　

　

c. What other ocular signs may be present in this condition?

　

In addition to severe ectropion, the following ocular changes may occur:

  • scarring and vascularization of the cornea from exposure

  • brush-like branching of the cornea nerves without thickening

  • cataract

　

e. How would you manage this patient?

　

The ocular irritation is secondary to exposure from the ectropion. So treatment should aim at the ocular surface and the skin.

  • Ocular surface: regular preservative-free eye drops to reduce the symptoms of dry eyes, wearing glasses outdoor also protect the eyes from dust and drying (those with shields).

  • Skin: Hydrate the skin with petrolatum and 10% urea cream.2 This will prevent worsening of the cicatricial ectropion.

If the above measures fail to improve the symptoms the patient may undergo ectropion operation with skin grafts. However, the recurrence rate is high.

　

Reference:

1. Mannis MJ, Macsai and Huntley AC. Eye and skin disease. Lippincott-Raven Publishers, Philadelphia, 1996.

　

2. Gregkin JL, Basler RS. New treatment for ichythyosis. Cutis 1980; 25: 432-434.