Peripheral ulcerative keratitis associated with chronic malabsorption syndrome and miliary tuberculosis in a child.

A 16-year-old girl presented with pain, redness, watering, and blurring of vision in her right eye. Slit lamp examination revealed the presence of peripheral ulcerative keratitis (PUK) and nodular scleritis. On clinical examination, the patient had stunted growth, low body mass index, and enlarged axillary nodes. Giardia cysts were present in the stool sample and histopathology of axillary lymph nodes showed caseating necrosis suggestive of tuberculosis (TB). A diagnosis of PUK with chronic malabsorption syndrome secondary to giardiasis and miliary TB was made. Oral metronidazole, anti-tubercular treatment, high protein diet and vitamin supplements were started. Topical steroids were started for peripheral ulcerative lesions following, which the PUK resolved.

Introduction

Peripheral ulcerative keratitis (PUK) is a potentially devastating disorder with crescent-shaped destructive inflammation at the margin of the corneal stroma. Clinical features include an epithelial defect, stromal inflammatory cells, progressive stromal degradation and thinning.[1] Systemic diseases that may cause PUK include collagen vascular diseases.[2] such as rheumatoid arthritis, Wegener granulomatosis, polyarteritis nodosa, relapsing polychondritis, and systemic lupus erythematosus. Apart from local infectious (e.g., herpes simplex keratitis, fungal keratitis), and noninfectious disorders (e.g., Mooren ulcer, marginal keratitis), systemic infectious conditions (e.g., hepatitis, syphilis, tuberculosis [TB]) may also predispose to PUK.

TB occurs in every part of the world, but is more common in developing nations. In 2012, the largest number of new TB cases occurred in Asia, accounting for 60% of new cases globally. There are a number of risk factors for TB infection such as HIV, Hodgkin lymphoma, end-stage renal disease, chronic lung disease, chronic malnutrition, and alcoholism.[3]

Giardia species are endemic in areas of the world that have poor sanitation.[4] In developing countries, the disease is an important cause of morbidity. Anorexia, fatigue, malaise, and weight loss are common. Weight loss occurs in more than 50% of patients and averages 10 pounds. Chronic illness may occur. Adults may present with long-standing malabsorption syndrome and children, with failure to thrive. This condition acts as a risk factor for new infection as well as reactivation of TB.

This case highlights the importance of ruling out risk factors for reactivation of TB in patients along with the role of complete systemic evaluation and management in the treatment of PUK.

Case Report

A 16-year-old girl presented to our cornea services with pain, redness, watering and blurring of vision in her right eye since last six months. Her local practitioner had prescribed her topical antibiotics and steroids but saw no improvement.

Past medical records showed an episode of pulmonary TB 1-year ago for which the patient had taken anti-tubercular treatment (ATT). Systemic examination revealed a severely stunted growth with a body mass index of 12.8 kg/m2. The enlarged spleen was palpable on abdominal examination. Matted, nontender lymph nodes were palpable in the right axilla. The patient had not yet achieved menarche.

On slit lamp biomicroscopy, the right eye had multiple areas (approximately 2 mm × 2 mm) of stromal thinning at the limbus associated with epithelial defect [Figure 1]. Iris tissue was adherent to the inferiorly thinned out cornea at 6’o clock. The pupil was vertically oval. Nodular scleritis was also present in the temporal quadrant. Lens and posterior segment examination were normal. The patient had a visual acuity of 20/60 improving to 20/30 with refraction. The fellow eye did not show any abnormality on examination.

Figure 1

Slit lamp photograph of the affected eye showing multiple areas of peripheral ulcerative keratitis

Blood investigations were suggestive of hypochromic anemia with hemoglobin (Hb) value of 8.9 g/dL and packed call volume value of 27.4%. Serum albumin and globulin levels were below the normal range. Erythrocyte sedimentation rate levels were raised with reading of 50 mm on Wintrobe testing. Stool sample microscopy showed multiple cyst of Giardia lamblia. Serum parathormone levels were raised with low Vitamin D levels. Follicle-stimulating hormone (FSH) and Luteinizing hormone (LH) were abnormally low. Axillary lymph node biopsy revealed caseating necrotizing granulomas positive for acid-fast bacilli on Ziehl–Neelsen stain. Ultrasonography abdomen revealed periportal and portocaval lymphadenopathy. All tests for collagen vascular diseases were negative. Scraping and culture from peripheral ulcerative lesions failed to detect any microorganism.

The patient was treated with oral metronidazole hydrochloride (5 mg/kg) tds for 5 days. High protein and carbohydrate diets were prescribed. Calcium Carbonate 60,000 units were prescribed along with Vitamin D supplements. ATT was restarted as per directly observed treatment,[5] short course protocol for miliary TB. Topical steroids were started 6 times a day along with moxifloxacin hydrochloride 0.5% tds.

After 1-month of treatment, patient showed an increase in appetite with modest weight gain. Hb and Vitamin D levels showed improvement. Nodular scleritis resolved [Figure 2] and PUK showed decreased activity with symptomatic relief.

Figure 2

Slit lamp photograph at 1-month follow-up showing resolution of nodular scleritis and healing ulcerative lesions

Discussion

G. lamblia is a common intestinal parasite and a frequent cause of diarrheal illness throughout the world.[4] The wide variety of clinical presentations, from severe disease to an asymptomatic carrier state, makes the definitive determination of pathogenesis difficult. Worldwide, the majority of patients infected with G. lamblia are asymptomatic. With chronic illness, malabsorption of fat, lactose, Vitamin A, and Vitamin B12 are reported, and failure of children to thrive has been noted.

In our patient, infection with G. lamblia had led to the chronic malabsorption syndrome. This resulted in stunted growth and failure to thrive. The patient had not attained menarche and had abnormally low levels of FSH/LH on testing. It also made the patient susceptible to recurrent TB activation. In spite of having received a full course of ATT the patient's systemic condition did not improve. Systemic TB is a known cause of PUK in adults, but its association with PUK in children is very rare.

Normally PUK is a crescent shaped destructive inflammation of the juxtalimbal corneal stroma, and the ulceration may progress both centrally and circumferentially. In our case, multiple areas of perilimbal thinning of PUK were present. This may be a pointer toward systemic etiology of the disease, as a local pathology would have a contiguous lesion.

Associated nodular scleritis is a highly significant finding because its presence signals an underlying systemic process. Our case highlights the fact that complete systemic evaluation is of utmost importance in the treatment of PUK.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.