Severe Bilateral Ectropion in Lamellar Ichthyosis: A Case Report

Area of Science:

• Dermatology
• Ophthalmology
• Genetics

Background:

• Lamellar ichthyosis is a rare genetic disorder causing severe skin scaling and dryness.
• Congenital ectropion and eclabium are common in lamellar ichthyosis, leading to ocular complications.
• Corneal exposure due to ectropion can result in ulceration, perforation, and phthisis bulbi.

Observation:

• A North African newborn presented with congenital lamellar ichthyosis, severe scaling, eclabium, and bilateral ectropion.
• Ophthalmologic and dermatologic collaboration was crucial for the infant's care.
• Treatment involved oral acitretin, skin emollients, lubricating eye drops/gels, and eyelid massage.

Findings:

• After six months, the treatment yielded excellent dermatologic and ophthalmologic outcomes.
• Early medical intervention for ectropion in lamellar ichthyosis is effective.
• Prompt management prevents progression to cicatricial ectropion and associated complications.

Implications:

• Medical management of ectropion in lamellar ichthyosis is feasible and highly effective when initiated early.
• This approach avoids surgical intervention and potential complications of eyelid fibrosis.
• Collaborative care between ophthalmologists and dermatologists is vital for optimal patient outcomes.