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KMID : 0360220070480070980
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Journal of the Korean Ophthalmological Society
2007 Volume.48 No. 7 p.980 ~ p.984
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A Case of Ocular Manifestation of Anhidrotic Ectodermal Dysplasia
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Lee Myung-Won
Lee Dong-Cho
Chang Moo-Hwan
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Abstract
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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.
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KEYWORD
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Anhidrotic ectodermal dysplasia, Dry eye syndrome, Entropion
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