---
title: Harlequin ichthyosis
description: "Harlequin ichthyosis is a severe congenital skin disorder causing thick, armour-like scales and ectropion at birth. Eye complications arise mainly from exposure and dryness. ## Ocular issues The tight skin pulls the lids away from the globe. - Marked ectropion…"
url: "https://bluefinvision.com/knowledge-base/topics/conditions-diseases/harlequin-ichthyosis/"
updated: "2025-12-16"
category: "Conditions & Diseases"
---

# Harlequin ichthyosis

Harlequin ichthyosis is a severe congenital skin disorder causing thick, armour-like scales and ectropion at birth. Eye complications arise mainly from exposure and dryness.

## Ocular issues

The tight skin pulls the lids away from the globe.

- Marked ectropion and lagophthalmos leading to exposure keratopathy
- Risk of corneal ulceration, scarring, and infection
- Difficulty closing eyes fully even during sleep
- Underlying ABCA12 gene mutations

## Management

Intensive early care can preserve vision.

- Frequent lubricating drops and ointment to protect the surface
- Moisture chambers, bandage lenses, or temporary tarsorrhaphy
- Dermatology-guided systemic retinoids and skin care
- Ongoing ophthalmic follow-up for scarring and refractive errors