Download Chronic Inflammation and Limbal Stem Cell Deficiency

The AOA’s CLCS Newsletter, June 2014
Chronic Inflammation and Limbal Stem Cell Deficiency
Randall McPherran, O.D.
A 7-year-old Hispanic female was referred to our rural health clinic by her
primary care physician (PCP) for ocular involvement of suspected atopic
dermatitis. She presented with significant rhinophyma, bilateral advanced
meibomian glands dysfunction and corneal involvement including pannus and
neovascularization.
Figures 1 and 2: Upper and lower lids revealing extensive erythema. Note area of bleeding on upper lid secondary to
patient scratching in response to intense itching. Photos courtesy of the author.
Figures 3 and 4: Upper and lower lids revealing extensive meibomian gland dysfunction. Note area of chalazion on
lower lid. Photos courtesy of the author.
Contained within the areas of neovascularization were focal areas of inflammation with an
appearance similar to Salzman's nodules.
The AOA’s CLCS Newsletter, June 2014
Figure 5: Neovascularization and limbal destruction that was evident bilaterally. Note Salzman's like inflammatory
nodules that later "resolved" to scar like formations with steroid treatment. Photo courtesy of the author.
Given her age, treatment with tetracyclines or doxycycline was initially withheld. The
patient responded well to lid hygiene treatment, omega-3 fatty acid supplements, topical
antibiotics and steroid treatment, presenting with significantly decreased inflammation
on follow-up.1 However, tapering the steroid resulted in a resumption of the
inflammatory cascade and continued limbal stem cell (LSC) involvement and
destruction. Though rare in Hispanics and children,1 ocular rosacea fit the presenting
profile and this diagnosis was later confirmed through the PCP.1,2
The patient was referred to a corneal specialist who confirmed the LSC involvement
both clinically and by impression cytology.3 Unfortunately, due to insurance and other
challenges, the patient presents only episodically at the corneal specialist, as well as
our rural clinic, and is frequently lost to follow-up despite all attempts to maintain the
child within the system over nearly a four-year period. The patient currently has a
significant bilateral LSC involvement/destruction. Under care of the corneal specialist,
the patient is being treated with Pred Forte BID OU, 4000 mg of flaxseed oil, extensive
lid hygiene and oral dicloxacillin.1
LSC deficiency will inhibit corneal epithelial self-renewal (which occurs approximately
every nine to 12 months), resulting in persistent epithelial defects. LSC deficiency can
be categorized into two major subdivisions: direct tissue destruction secondary to
chemical, thermal, UV or other trauma, or the second category involving inflammatory
damage such as aniridia, ocular pemphigoid, VKC or ocular rosacea.3 While limbal stem
cell transplantation has been increasingly successful in treating direct tissue damage,
patients with underlying immunologically driven LSC deficiency offer a significantly
poorer prognosis for successful outcome.1,2,5 This is particularly worrisome for the
young patient in this case presentation.
References:
1) Oltz M, Check J. Rosacea and its ocular manifestations. Optometry (2011) 82, 92-103.
2) Samson C, Nduaguba C. Limbal Stem Cell Transplantation in Chronic Inflammatory Eye Disease. Ophthalmology
2002;109:862–868
The AOA’s CLCS Newsletter, June 2014
3)
4)
5)
Sangwan V, et al. Vernal Keratoconjunctivitis With Limbal Stem Cell Deficiency. Cornea Volume 30, Number 5, May
2011.
Rama P et al. Limbal Stem-Cell Therapy and Long-Term Corneal Regeneration. N Engl J Med 2010;363:147-55
Ahmad S. Concise Review: Limbal Stem Cell Deficiency, Dysfunction, and Distress. Stem Cells Trans Med 2012, 1:110115.
Dr. McPherran is an Associate Clinical Professor and chief of UC Berkeley Eye
Services at Castle Family Health Center. He is a Fellow of the American Academy of
Optometry and has served as past chief examiner to the National Board of Examiners in
Optometry.
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