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In the Context of Cicatricial Disease

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

A woman in her 60s with a history of hypothyroidism presented with a 6-month history of progressive, diffuse redness and tearing in the left eye. She maintained a healthy diet and denied any history of malnutrition, gastrointestinal procedures, recent ocular infections, or chemical exposures. Her ocular history was notable for cataract extraction with intraocular lens implantation in both eyes. Shortly after cataract surgery in the left eye, performed 1 year prior to presentation, the patient developed cystoid macular edema treated with a sub-Tenon injection of triamcinolone acetonide. Ocular hypertension was noted after corticosteroid treatment, and the patient started brimonidine-timolol drops twice daily in the left eye.

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Ocular surface squamous neoplasia (OSSN) in the context of cicatricial disease

B. Test for vitamin A deficiency, with additional conjunctival biopsies for pathologic evaluation and testing for immune deposits

This case demonstrates an atypical presentation of OSSN. The bilateral cicatrizing disease prompted workup for other underlying conditions, such as vitamin A deficiency and ocular cicatricial pemphigoid (OCP). Brimonidine-timolol drops were discontinued because medicamentosa is a common cause of cicatrizing disease.1 Discontinuation of the offending medication is often diagnostic; however, the severity of the keratinization and the bilaterality (brimonidine-timolol drops were used only unilaterally) suggested that medicamentosa alone was not the culprit (choice C). Furthermore, these features were concerning for an underlying life-threatening cicatricial process, such as mucous membrane pemphigoid, making observation inappropriate (choice A).2 It would likewise be inappropriate to perform lesion excision with tissue disposal (choice D). Vitamin A levels returned as normal, and the patient’s medical history did not suggest a nutritional deficiency or malabsorption.

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Article Information

Corresponding Author: Zeba A. Syed, MD, Cornea Service, Wills Eye Hospital, 840 Walnut St, Ste 920, Philadelphia, PA 19107 (zebaasyed@gmail.com).

Published Online: August 27, 2020. doi:10.1001/jamaophthalmol.2020.1606

Conflict of Interest Disclosures: None reported

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Kirzhner  M , Jakobiec  FA .  Ocular cicatricial pemphigoid: a review of clinical features, immunopathology, differential diagnosis, and current management.   Semin Ophthalmol. 2011;26(4-5):270-277. doi:10.3109/08820538.2011.588660PubMedGoogle ScholarCrossref
2.
Choi  CJ , Jakobiec  FA , Zakka  FR , Foster  CS , Chodosh  J , Freitag  SK .  Conjunctival squamous cell neoplasia associated with ocular cicatricial pemphigoid.   Ophthalmic Plast Reconstr Surg. 2017;33(6):e157-e160. doi:10.1097/IOP.0000000000000926PubMedGoogle ScholarCrossref
3.
Faraj  HG , Hoang-Xuan  T .  Chronic cicatrizing conjunctivitis.   Curr Opin Ophthalmol. 2001;12(4):250-257. doi:10.1097/00055735-200108000-00003PubMedGoogle ScholarCrossref
4.
Anzaar  F , Cabrita  F , Ahmed  M , Foster  CS .  The frequency of other autoimmune disorders in patients with ocular cicatricial pemphigoid.   Acta Ophthalmol. 2012;90(3):e253-e254. doi:10.1111/j.1755-3768.2011.02174.xPubMedGoogle ScholarCrossref
5.
Heiligenhaus  A , Schaller  J , Mauss  S ,  et al.  Eosinophil granule proteins expressed in ocular cicatricial pemphigoid.   Br J Ophthalmol. 1998;82(3):312-317. doi:10.1136/bjo.82.3.312 PubMedGoogle ScholarCrossref
6.
Thomas  BJ , Galor  A , Nanji  AA ,  et al.  Ultra high-resolution anterior segment optical coherence tomography in the diagnosis and management of ocular surface squamous neoplasia.   Ocul Surf. 2014;12(1):46-58. doi:10.1016/j.jtos.2013.11.001PubMedGoogle ScholarCrossref
7.
Joag  MG , Sise  A , Murillo  JC ,  et al.  Topical 5-fluorouracil 1% as primary treatment for ocular surface squamous neoplasia.   Ophthalmology. 2016;123(7):1442-1448. doi:10.1016/j.ophtha.2016.02.034PubMedGoogle ScholarCrossref
8.
Shields  JA , Shields  CL , De Potter  P .  Surgical management of conjunctival tumors: the 1994 Lynn B. McMahan Lecture.   Arch Ophthalmol. 1997;115(6):808-815. doi:10.1001/archopht.1997.01100150810025PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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