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JAMA Ophthalmology Clinical Challenge
June 2018

Bilateral Cicatrizing Conjunctivitis in a Middle-aged Man

Author Affiliations
  • 1Department of Ophthalmology & Visual Science, Yale School of Medicine, New Haven, Connecticut
  • 2Department of Pathology, Yale School of Medicine, New Haven, Connecticut
JAMA Ophthalmol. 2018;136(6):706-707. doi:10.1001/jamaophthalmol.2017.5490
Case

A 53-year-old man presented with chronic ocular irritation that he attributed to his eyelashes, which he usually removed himself. His medical history was significant for hypertension and multiorgan sarcoid that was confirmed in his late 20s by a sinus biopsy demonstrating consistent pathologic findings. The patient reported wearing contact lenses daily. His best-corrected visual acuity was 20/25 OD and 20/30 OS. Results of his eye examination were notable for bilateral trichiasis of the lower eyelids with scarring of the inferior fornices and symplepharon (Figure 1), but there was no evidence of intraocular involvement of sarcoid. He denied any skin blisters, oral ulcers, difficulty swallowing, trauma, severe conjunctivitis, or seasonal allergies. He was taking 160 mg of valsartan and 10 mg of prednisone daily since 2006 and had recently started taking 20 mg of methotrexate sodium weekly. The patient had a history of parental smoke exposure as a child and a 20 pack-year personal smoking history. He worked with high-temperature coating on aircraft components and reported exposure to aluminum, nickel, and cobalt but denied exposure to beryllium. Epilation was performed during his visit.

Figure 1.
A, Scarring of inferior fornix of left lower eyelid (arrowhead). B, Symblepharon of right lower palpebral conjunctiva.

A, Scarring of inferior fornix of left lower eyelid (arrowhead). B, Symblepharon of right lower palpebral conjunctiva.

Box Section Ref ID

What Would You Do Next?

  1. Change therapy from methotrexate to mycophenolate mofetil

  2. Increase the prednisone dose to 15 mg daily

  3. Refer patient for surgical correction of trichiasis

  4. Arrange for conjunctival biopsy with immunostaining

Discussion
Diagnosis

Bilateral cicatrizing conjunctivitis secondary to sarcoidosis

What to Do Next

D. Arrange for conjunctival biopsy with immunostaining

Given the patient’s presenting symptom of chronic ocular irritation as well as histories of multiorgan sarcoid and exposure to heavy metals, ocular cicatricial pemphigoid, a subtype of mucous membrane pemphigoid, IgG4-related ophthalmic disease, drug-induced cicatrizing conjunctivitis, and ocular sarcoidosis should all be included in the differential diagnosis. Although the involvement of the lower tarsal conjunctiva and inferior fornix, as well as the lack of oral mucosal lesions or skin lesions, favor drug-induced cicatrizing conjunctivitis rather than ocular cicatricial pemphigoid, drug-induced cicatrizing conjunctivitis is more often associated with the use of topical glaucoma medications, of which this patient had no history.1

Conjunctival biopsy with immunostaining (choice D) should be performed to distinguish between ocular cicatricial pemphigoid, IgG4-related ophthalmic disease, and ocular sarcoid. Even though biopsy of intraocular tissue is still uncommonly performed, pathologic confirmation of the diagnosis is recommended before initiation of treatment or change in current therapy, such as switching the patient to mycophenolate mofetil (choice A) or increasing the prednisone dose to 15 mg (choice B).2 Although bilateral trichiasis was noted on examination, it is not associated with forniceal scarring or symblepharon, making surgical correction of trichiasis (choice C) incorrect.

Sarcoidosis is an idiopathic, multisystemic, chronic inflammatory disease characterized by epithelioid noncaseating granulomas on histopathologic findings, with ophthalmologic manifestations present in 25% to 80% of patients.3 Anterior uveitis, seen in up to 65% of patients with ophthalmologic involvement, is the most common manifestation of ocular sarcoidosis, followed by conjunctival nodules in 40% of patients.4,5 However, up to one-third of patients with anterior uveitis will remain asymptomatic.4 The eye may be the main site of disease without any systemic involvement. It is recommended that all patients diagnosed with sarcoidosis undergo slit-lamp examination and ophthalmoscopic examination irrespective of ocular symptoms.6

Cicatrizing conjunctivitis, typically characterized by subepithelial fibrosis early in the disease and subsequent forniceal foreshortening with symblepharon formation, has been reported in a small number of case reports as an ocular manifestation of sarcoidosis.7-9 More commonly, conjunctival involvement in the form of conjunctival nodules resembling follicular conjunctivitis has been reported in patients with sarcoidosis.10 Although rare, this case emphasizes that sarcoidosis should be considered in the differential diagnosis of cicatrizing conjunctivitis. Given that sarcoidosis is associated with an increase in morbidity and mortality and that ocular sarcoidosis can lead to blindness, its prompt diagnosis and treatment is critical.7

Patient Outcome

The patient returned for a conjunctival biopsy, which was obtained from the inferior fornix of the left eye. Histopathologic examination revealed subepithelial noncaseating granulomas (Figure 2), while results of IgG, IgA, IgM, C3, C1q, acid-fast bacillus, and silver stains were negative. In addition, no foreign material was identified by polarizing microscopy. The noncaseating granulomas, given the prior diagnosis of sarcoidosis, were consistent with ocular sarcoidosis. The patient continued to take 10 mg of prednisone daily and 20 mg of methotrexate sodium weekly for treatment of sarcoidosis. At 3.5 weeks after presentation, examination revealed stable bilateral trichiasis and inferior forniceal shortening and no signs of active inflammation. The patient’s pulmonary and cardiac findings were stable.

Figure 2.
Conjunctival biopsy revealing noncaseating granuloma (arrowhead) (hematoxylin-eosin, original magnification ×10).

Conjunctival biopsy revealing noncaseating granuloma (arrowhead) (hematoxylin-eosin, original magnification ×10).

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Article Information

Corresponding Author: Nicholas Apostolopoulos, BA, Department of Ophthalmology & Visual Science, Yale School of Medicine, 123 York St, Apt 18J, New Haven, CT 06511 (nicholas.apostolopoulos@yale.edu).

Published Online: March 29, 2018. doi:10.1001/jamaophthalmol.2017.5490

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

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

References
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