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Eyelid abnormalities in lamellar ichthyoses.

A A Cruz1, F A Menezes, R Chaves

  • 1Department of Ophthalmology of School of Medicine of Ribeirão Preto, São Paulo, Brazil.

Ophthalmology
|October 3, 2000
PubMed
Summary
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Lamellar ichthyoses (LI) can cause severe eyelid abnormalities, including lagophthalmos and ectropion. Corneal damage and vision loss may occur even without eyelid ectropion in patients with LI.

Area of Science:

  • Ophthalmology
  • Dermatology
  • Genetics

Background:

  • Lamellar ichthyoses (LI) are a group of rare genetic skin disorders characterized by scaling of the skin.
  • Eyelid abnormalities are a known complication of LI, but their spectrum and relationship with corneal damage require further elucidation.

Purpose of the Study:

  • To describe the spectrum of eyelid abnormalities in patients with lamellar ichthyoses.
  • To investigate the association between eyelid ectropion and corneal damage in LI patients.

Main Methods:

  • Retrospective observational case series.
  • Reviewed eyelid and corneal examinations of 10 patients with LI (8 classic LI, 2 congenital ichthyosiform erythroderma).
  • Analyzed the relationship between eyelid ectropion and corneal damage.

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Main Results:

  • All patients exhibited cicatricial lagophthalmos.
  • Ectropion was present in 6/8 classic LI patients; 2/8 had no ectropion.
  • Corneal damage leading to vision loss occurred in 3/8 classic LI patients, with 2 of these lacking upper eyelid ectropion.

Conclusions:

  • Severe corneal damage can manifest in LI irrespective of the presence of upper or lower eyelid ectropion.
  • Ophthalmological monitoring is crucial for LI patients to detect and manage potential corneal complications early.