J Korean Ophthalmol Soc.  2007 Jul;48(7):980-984.

A Case of Ocular Manifestation of Anhidrotic Ectodermal Dysplasia

Affiliations
  • 1Department of Ophthalmology, Dankook University College of Medicine, Cheonan, Korea. cataract@empal.com

Abstract

PURPOSE: This clinical report describes the characteristics and ophthalmic treatment of a patient with anhidrotic ectodermal dysplasia showing bilateral upper eyelid entropion, conjunctival mass and dry eye syndrome.
METHODS
A 12-year-old boy presented to our clinic complaining of a white mass on the lower tarsal conjunctiva, foreign body sensation, and an itching in his right eye of one-year duration. He had developmental defects in his hair, teeth, nails and sweat glands. He had dry skin, a flat upper-lip, heat intolerance, a saddle-backed nose, and hypohidrosis. Ophthalmic examination showed blepharitis, a conjunctival mass, and bilateral upper eyelid entropion.
RESULTS
Upper eyelid entropion surgery was performed. An excisional biopsy of the conjunctival mass showed nonspecific acute and chronic inflammation.
CONCLUSIONS
We concluded that the conjunctival mass developed as a result of chronic inflammation caused by chronic conjunctivitis and dry eye syndrome. Regular lid-hygiene with swab and lubrication is useful to minimize recurrent ocular inflammation in anhidrotic ectodermal dysplasia.

Keyword

Anhidrotic ectodermal dysplasia; Dry eye syndrome; Entropion

MeSH Terms

Biopsy
Blepharitis
Child
Conjunctiva
Conjunctivitis
Dry Eye Syndromes
Ectodermal Dysplasia*
Entropion
Eyelids
Foreign Bodies
Hair
Hot Temperature
Humans
Hypohidrosis
Inflammation
Lubrication
Male
Nose
Pruritus
Sensation
Skin
Sweat Glands
Tooth
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