Rare Ocular Association With Hailey-Hailey Disease

Hailey-Hailey disease is an autosomal dominant blistering disorder characterized by junctional abnormalities of epidermal keratinocytes. Vesiculobullous eruptions affect the intertriginous areas of individuals with the condition. Ocular involvement associated with Hailey-Hailey disease is rare.

A 12-year-old female with a history of recurrent blisters since the age of 3 years presented with concurrent redness, irritation, and watering of both eyes. Slit lamp examination revealed bilateral conjunctival congestion, peripheral corneal neovascularization, lack of corneal luster, and corneal haze. The ocular signs were more prominent in the left eye. Important differentials considered were pemphigus vulgaris and allergic conjunctivitis. However, lack of clinical signs of allergic conjunctivitis, lack of involvement of the oral mucosa, the classic distribution of blisters and their aggravation by triggering factors, the absence of Nikolsky sign, and biopsy of a skin lesion ruled out these 2 diagnoses. The patient was treated with low-potency steroid eye drops, an ocular lubricant, and eye ointment. At her 2-week follow-up examination, the patient exhibited decreased conjunctival congestion, improved corneal luster, and symptomatic relief.

Ocular involvement in Hailey-Hailey disease can lead to chronic ocular inflammation and sequelae, causing a decrease in vision. Our case is noteworthy because of the early onset of Hailey-Hailey disease and the associated ocular manifestations.