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JAMA Ophthalmology Clinical Challenge
December 26, 2019

Infectious Keratitis in a 23-Year-Old Patient With Netherton Syndrome

Author Affiliations
  • 1Duke Eye Center, Department of Ophthalmology, Duke University, Durham, North Carolina
  • 2Duke Eye Center, Department of Pathology and Ophthalmology, Duke University, Durham, North Carolina
JAMA Ophthalmol. 2020;138(2):210-211. doi:10.1001/jamaophthalmol.2019.4920

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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    1 Comment for this article
    Interesting case
    Muna Malik, M.PHIL Microbiology | Post Graduate Medical Institute, Ameer Ud din Medical college, Lahore General Hospital, Lahore Pakistan.
    A very interesting and rare case report is discussed. Netherton's syndrome is a rare syndrome that is encountered in dermatology, especially in developing countries like Pakistan. A few cases of Netherton's syndrome were reported in Pakistan [1]. The keratitis of this rare syndrome is often underevaluated in such developing countries; no proper diagnosis is made for proper and prompt treatment of this curable disease. Though PCR and culture and sensitivity for viral and bacterial infections of the cornea can be done scrapings, clinicians start empirical therapy and no investigation is done for proper sensitive drug therapy. As a result, therapy resistance and treatment failure occurs.

    1. Khan IU, Chaudhary R. Netherton's Syndrome, an uncommon genodermatosis. Journal of Pakistan Association of Dermatology. 2017 Jan 3;16(3):180-2.