Kindler syndrome causing severe cicatricial ectropion

Area of Science:

• Ophthalmology
• Genetics
• Dermatology

Background:

• Kindler syndrome is a rare genodermatosis characterized by photosensitivity, poikiloderma, and skin fragility.
• Ocular manifestations, including cicatricial ectropion and keratopathy, significantly impact vision and quality of life.

Observation:

• A 32-year-old female with Kindler syndrome presented with a 5-year history of bilateral lower eyelid ectropion, retraction, and tarsal eversion.
• Severe anterior lamellar cicatricial changes led to corneal exposure and recurrent erosions, refractory to conservative management.

Findings:

• Surgical correction of the lower eyelid malposition resulted in the resolution of corneal disease.
• The patient experienced improved lower eyelid position and significant relief from ocular surface symptoms post-surgery.

Implications:

• This case highlights the efficacy of surgical management for complex eyelid deformities in Kindler syndrome.
• Oculoplastic surgeons play a crucial role in managing the ocular complications associated with Kindler syndrome, improving patient outcomes.