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A 23-year-old white man with Netherton syndrome, a skin condition of ichthyosiform erythroderma, presented to the clinic with what he described as “pressure behind the eye” and visual acuity “going in and out” in his left eye. The patient had an ocular history of herpes simplex (HSV) keratitis with a previous perforation of the right eye repaired with a patch graft, advanced open-angle glaucoma in the right eye, and ocular hypertension of the left eye, and he was receiving brimonidine 3 times a day in both eyes and acetazolamide, 250 mg, 3 times a day.
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Herpes simplex keratitis with bacterial superinfection
C. Start oral antiviral and topical antibiotic therapy
Netherton syndrome is an autosomal-recessive condition. It causes excessive shedding of skin, atopic eczema, pruritus, and fragile hair, including eyelashes and eyebrows.1
The clinical manifestations of Netherton syndrome may necessitate consideration of causes of dendritic corneal lesions, including HSV, epithelial regeneration lines, neurotrophic keratopathy, varicella zoster virus, iatrogenic causes, and infestations with Acanthamoeba species. Given this patient’s history and presentation, HSV was most likely.
While, to our knowledge, the association of Netherton syndrome and HSV has not been documented previously, there is a well-established connection between atopy and ophthalmic HSV. Patients with atopy have been shown to be twice as likely to develop ophthalmic HSV as control individuals.2 Severe primary, recurrent, and bilateral disease are more common in patients with atopy than unaffected patients.3,4 Similarly, in patients with ocular HSV, the odds ratio of having atopic disease is 1.5 to 4.8 compared with control individuals.5 Patients with atopy have been reported to have more episodes of infectious HSV than inflammatory episodes compared with those without atopy.6
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Corresponding Author: Mark F. Goerlitz-Jessen, MD, Duke Eye Center, Department of Ophthalmology, Duke University, 2351 Erwin Rd, Durham, NC 27710 (email@example.com).
Conflict of Interest Disclosures: None reported.
Published Online: December 26, 2019. doi:10.1001/jamaophthalmol.2019.4920
Additional Contributions: We thank the patient for granting permission to publish this information.
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