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

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

Corresponding Author: Mark F. Goerlitz-Jessen, MD, Duke Eye Center, Department of Ophthalmology, Duke University, 2351 Erwin Rd, Durham, NC 27710 (mark.goerlitz.jessen@duke.edu).

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.

References
1.
US National Library of Medicine. Genetics home reference: Netherton syndrome. https://ghr.nlm.nih.gov/condition/netherton-syndrome. Accessed May 2019.
2.
Borkar  DS, Gonzales  JA, Tham  VM,  et al.  Association between atopy and herpetic eye disease: results from the Pacific Ocular Inflammation Study.  JAMA Ophthalmol. 2014;132(3):326-331. doi:10.1001/jamaophthalmol.2013.6277PubMedGoogle ScholarCrossref
3.
Arslanagić  N, Arslanagić  R.  [Netherton syndrome with recurrent herpes of facial skin]  [in Croatian].  Med Arh. 2002;56(4):221-224.PubMedGoogle Scholar
4.
Easty  D, Entwistle  C, Funk  A, Witcher  J.  Herpes simplex keratitis and keratoconus in the atopic patient. a clinical and immunological study.  Trans Ophthalmol Soc U K. 1975;95(2):267-276.PubMedGoogle Scholar
5.
Prabriputaloong  T, Margolis  TP, Lietman  TM, Wong  IG, Mather  R, Gritz  DC.  Atopic disease and herpes simplex eye disease: a population-based case-control study.  Am J Ophthalmol. 2006;142(5):745-749. doi:10.1016/j.ajo.2006.06.050PubMedGoogle ScholarCrossref
6.
Rezende  RA, Hammersmith  K, Bisol  T,  et al.  Comparative study of ocular herpes simplex virus in patients with and without self-reported atopy.  Am J Ophthalmol. 2006;141(6):1120-1125. doi:10.1016/j.ajo.2006.01.072PubMedGoogle ScholarCrossref
7.
Azher  TN, Yin  XT, Tajfirouz  D, Huang  AJ, Stuart  PM.  Herpes simplex keratitis: challenges in diagnosis and clinical management.  Clin Ophthalmol. 2017;11:185-191. doi:10.2147/OPTH.S80475PubMedGoogle ScholarCrossref
8.
McGilligan  VE, Moore  JE, Tallouzi  M,  et al.  A comparison of the clinical and molecular diagnosis of herpes simplex keratitis.  Open J Ophthalmol. 2014;4(3):65-74. doi:10.4236/ojoph.2014.43011Google ScholarCrossref
9.
Pramod  NP, Thyagarajan  SP, Mohan  KV, Anandakannan  K.  Polymerase chain reaction in the diagnosis of herpetic keratitis: experience in a developing country.  Can J Ophthalmol. 2000;35(3):134-140. doi:10.1016/S0008-4182(00)80006-XPubMedGoogle ScholarCrossref
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