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Rare Ocular Association With Hailey-Hailey Disease.
Mamta Singh1, Yashdeep Singh Pathania2, Praggya Mishra1
1Department of Ophthalmology, All India Institute of Medical Sciences Rajkot, Rajkot, Gujarat, India.
Ochsner Journal
|June 20, 2025
Summary
Hailey-Hailey disease, a rare blistering disorder, can affect the eyes. This case highlights early-onset ocular symptoms in a pediatric patient, emphasizing the importance of recognizing these manifestations.
Area of Science:
- Dermatology
- Ophthalmology
Background:
- Hailey-Hailey disease is an autosomal dominant blistering disorder affecting epidermal keratinocytes.
- It typically presents with vesiculobullous eruptions in intertriginous areas.
- Ocular involvement is an uncommon manifestation.
Observation:
- A 12-year-old female with a history of recurrent blisters presented with bilateral eye redness, irritation, and watering.
- Slit lamp examination revealed conjunctival congestion, peripheral corneal neovascularization, reduced corneal luster, and corneal haze.
- Ocular signs were more pronounced in the left eye.
Findings:
- Differential diagnoses including pemphigus vulgaris and allergic conjunctivitis were excluded based on clinical presentation and biopsy.
- The patient received treatment with topical corticosteroids, lubricants, and ointment.
- Follow-up demonstrated reduced conjunctival congestion, improved corneal luster, and symptomatic relief.
Implications:
- This case underscores the rare but significant ocular manifestations of Hailey-Hailey disease.
- Early recognition and management of ocular symptoms are crucial to prevent vision-impairing sequelae.
- The early onset in this pediatric case highlights the need for heightened awareness in younger patients.
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