vitamin a deficiency is the leading cause of preventable childhood blindness in the developing world .
although rare in the united states , vitamin a deficiency has been known to occur as a result of poor dietary intake , liver diseases , and gastrointestinal malabsorption [ 2 , 3 , 4 , 5 ] .
vitamin a is a fat - soluble vitamin ingested in the diet in two forms : as retinol itself from animal sources , such as milk , meat , fish , liver , and eggs , or as the provitamin carotene from plant sources , such as green leafy vegetables , yellow fruits , and red palm oil . on the ocular surface ,
vitamin a deficiency has a wide range of ocular manifestations including conjunctival and corneal xerosis , keratomalacia , retinopathy , visual loss , and nyctalopia , also called night blindness , which is the earliest and most common symptom .
we report a case of bilateral sequential corneal ulceration in a patient with severe vitamin a deficiency in the context of eosinophilic gastroenteropathy .
a 29-year - old man was referred to our cornea unit with a left corneal ulcer of 6 weeks ' duration .
he was previously diagnosed with eosinophilic gastroenteropathy ( biopsy proven ) , skin atopy and atopic keratoconjunctivitis .
he had been treated with peroral steroids 15 mg / day and methotrexate 6 mg / week since the age of 11 years for his gastroenteropathy .
he had surgery for steroid - induced cataracts with toric intraocular lens implantation at the age of 27 years .
in addition , he had a number of food intolerances and subsisted mostly on a diet of potatoes . prior to his presentation , he had noted irritation , itchiness , and a decrease in vision in his left eye for 6 weeks and was treated with topical anti - allergic and topical steroid drops ; however , no improvement could be observed .
polymerase chain reaction testing for herpes simplex virus was negative , and the cultures did not show any growth .
acuity without correction was 20/80 od , improved by pinhole to 20/50 , and 20/150 os with a manifest refraction of 0.25/-2.5/5 , not improved by pinhole .
the intraocular pressure was 19 mm hg od and 21 mm hg os by tonopen .
slit - lamp examination showed temporal wrinkling of the conjunctiva in both eyes , with a dry granular pattern and loss of transparency ( fig . 

the left temporal cornea showed an epithelial defect overlying a partial - thickness ulcer with approximately 50% thinning and adjacent limbitis . in both eyes , the anterior chambers were quiet and there were well - centered posterior chamber iols .
our patient was initially diagnosed with peripheral ulcerative keratitis in the left eye in the presence of atopic keratoconjunctivitis .
his steroids were increased to 50 mg / day and tapered every 3 days by 10 mg / day .
he was put on preservative - free artificial tears . a vasculitic workup ( c - reactive protein ,
erythrocyte sedimentation rate , antinuclear antibody , antineutrophil cytoplasmic antibody , rheumatoid factor , syphilis serology ) was unremarkable .
serum was sent for analysis of vitamin a and retinol - binding protein ( rbp ) levels , with a processing time of several weeks at our location .
the ulcer in the left eye epithelialized over the following 2 weeks and subsequently vascularized from the temporal conjunctiva .
two weeks later , the patient presented to our clinic again with significant right eye pain .
he had a corneal ulcer in his right eye , complicated by a small corneal perforation , with the iris plugging the wound ( fig . 

serendipitously , at that time , the results of his serum vitamin a levels were available , revealing that the levels were significantly decreased to 0.2 mol / l ( normal range 0.9 - 2.5 ) . the rbp level was also reduced to 12 mg / l ( normal 30 - 92 ) .
the patient was treated with iv multivitamin ( containing 10,000 iu vitamin a , c , d , e and b complex ) for 5 consecutive days while in hospital , and then with oral vitamin a 200,000 iu once a week for 2 weeks .
2b ) and the corneal keratinization in both eyes resolved over the following 6 weeks ( fig .
we present a rare case of vitamin a deficiency secondary to a malnutrition syndrome leading to bilateral xerosis and sequential corneal ulceration .
similar reports from developed populations are rare , but have been described in association with disorders such as chronic alcoholism , cystic fibrosis , and anorexia nervosa .
the initial treatment with an increase of systemic steroids improved the ulceration and seemed to suggest an immunogenic process .
however , the involvement of the second eye while on steroid treatment forced consideration of other causes . clinical suspicion of xerophthalmia at the time of presentation prompted testing for vitamin a and serum rbp .
it was unfortunate in our case that the progression of the disease occurred in the time taken for results to be reported .
the earliest ocular symptom of vitamin a deficiency is nyctalopia , secondary to depletion of photopigments .
unfortunately , we were not able to obtain an electroretinogram ( erg ) before the treatment .
an abnormal erg at presentation in association with the anterior segment findings would have helped to establish a diagnosis of vitamin a deficiency before serum levels were available .
it has been shown that eosinophilic gastroenteropathy can cause malabsorption or protein - losing enteropathy [ 11 , 12 ] , but in our case , a gastroenterology consult confirmed inactive disease and showed no other signs of malabsorption ( normal albumin levels and international normalized ratio ) . the most likely reason was felt to be malnutrition due to lack of vitamin a uptake secondary to the patient 's multiple food intolerances that accompany eosinophilic gastroenteropathy .
the long - lasting success of oral vitamin a supplementation further favors previous malnutrition over malabsorption .
the oral dosage regimen is 200,000 iu vitamin a in oil , followed the next day with an additional dose of 200,000 iu .
if patients have severe corneal disease or malabsorption , the preferred dose is 100,000 iu water - miscible vitamin a administered intramuscularly .
it is important to note that if vitamin a deficiency is associated with a protein - energy malnutrition syndrome , protein levels must be corrected at the same time as vitamin supplementation .
our case serves as a reminder to corneal specialists in developed populations of the potentially devastating effects of vitamin a deficiency on the eye .
vitamin a deficiency should be suspected in any practice in the presence of corneal ulceration in combination with malabsorption and malnutrition syndromes .
 purpose : vitamin a deficiency is a very rare condition in the developed world and can lead to a variety of ocular changes from xerosis and xerophthalmia to corneal ulcer and perforation . 
 the treatment of this devastating disease is simple and inexpensive . 
 it is therefore important to recognize and treat accordingly , especially in the event of ulcers unresponsive to treatment or in the presence of severe malnutrition / malabsorption syndromes . 
 the purpose of this case report is to remind physicians of the potentially devastating effects of vitamin a deficiency on the eyes and to demonstrate outcomes after vitamin a treatment . 
 methods : single observational case report . 
 results : a 29-year - old male with known eosinophilic gastroenteropathy was treated with oral steroids for peripheral ulcerative keratitis . 
 two weeks after resolution , the patient suffered from peripheral ulcerative keratitis in his other eye , with a self - sealing perforation . 
 vitamin a deficiency was confirmed and successfully treated , leading to subsequent resolution of signs and symptoms . 
 conclusions : vitamin a deficiency can be present in patients with malabsorption and malnutrition syndromes and should be considered as cause of corneal ulceration . 
Introduction
Case Report
Results
Discussion
Disclosure Statement