Abstract
Background
The number of Hansen’s disease cases in Latin America and the Caribbean has decreased in the last decade; nevertheless, the region is still struggling with infections caused by Mycobacterium leprae. This is a case report that portrays the diagnostic and management challenges associated with atypical uveitic glaucoma that is due to Hansen’s disease.
Case presentation
A 62-year-old female was referred with a 2-year history of anterior uveitis of unknown etiology and ocular hypertension. Past medical history and general physical examination were unremarkable. Upon ocular examination, her best-corrected visual acuity (BCVA) was 20/25 in the OD and 20/60 in the OS. Tonometry showed intraocular pressures (IOPs) of 29 mmHg and 22 mmHg in her right and left eyes, respectively. The slit-lamp examination showed clinical signs of bilateral granulomatous anterior uveitis and cataracts; gonioscopy revealed open angles with some peripheral anterior synechiae for both eyes. Fundus examination and glaucoma tests revealed mild glaucomatous damage in the right eye. Given the presentation of uveitis, the respective questionnaire was completed by internal medicine and rheumatology. Four months later, after bilateral cataract surgery, the patient developed skin plaques on the face, neck, upper back, and extremities, which were biopsied and identified as positive for tuberculoid leprosy.
Conclusion
This is the first case report in Ecuador of atypical glaucoma triggered by infectious uveitis produced by Mycobacterium leprae . We describe a female patient’s clinical presentation with several ocular signs of leprosy and other nonspecific and rarely seen symptoms. Uveitis is a condition that often requires a multidisciplinary team of ophthalmologists and clinicians because of the possible manifestation of an underlying systemic disease, creating a challenge for all the medical personnel involved in the management of the case.
1
Background
Leprosy, one of the most common diseases documented throughout human history, is still present among patients with ocular morbidities. As Hansen stated, “there is no disease which so frequently gives rise to disorders of the eye as leprosy does”. The microorganism responsible for causing leprosy is Mycobacterium leprae , an acid-fast-stained bacilli, discovered by Gerhard Henrik Armauer Hansen in 1874. This disease, previously attributed to witchcraft, was responsible for millions of deaths before the arrival of antibiotics. The main features of this disease are skin ulcers, lack of skin sensitivity, muscle weakness, destruction of the nasal appendix, absence of hair on the eyebrows and eyelashes, changes in pigmentation, and diffuse involvement of the facial skin causing leonine facies, peripheral nervous system alterations and upper respiratory tract mucosa and eyes affections. ,
This infection is transmitted via airborne droplets of infected individuals; however, there have been reports of trauma-related transmission and zoonotic cases, which were the result of contact with armadillos and environmental reservoirs such as water sources. , Mycobacterium leprae replicates at temperatures of approximately 30 °C; therefore, it has a preference for low-temperature body areas, such as the peripheral nervous system, musculoskeletal system, upper respiratory tract, skin, mucosa, testicles, and anterior chamber of the eye. , , Because of the deformities and disabilities associated with the infection, patients throughout history have suffered from discrimination and stigmatization. In fact, the name leprosy derives from the Latin word lepros, which means defilement.
Leprosy is a disease that has been forgotten because of its low prevalence. In 2015, World Health Organization (WHO) reported 176,176 cases, calculating a prevalence of 0.2 cases per 10,000 people. In Ecuador, which is located in South America, the incidence reported in 2019 was less than 1 case per 100,000 persons, and was mainly concentrated in the Amazonian region of the country. In 2015, there were 178 new cases in the United States, predominantly in the states of Arkansas, California, Florida, Hawaii, Louisiana, New York, and Texas. Centers for Disease Control and Prevention (CDC) estimates that 5000 people in the United States have been cured but suffer from long-term sequelae such as blindness.
The Ridley and Jopling classification takes into account clinical, pathological, bacilloscopic, and immunological criteria to classify leprosy into six forms: tuberculoid (TT), borderline tuberculoid (BT), mid-borderline (BB), borderline lepromatous (BL), lepromatous (LL), and indeterminate (I). , , Lepromatous leprosy patients tend to have more ocular complications and vision impairment (p = .037) than patients with tuberculoid leprosy, borderline tuberculoid, and indeterminate leprosy.
The WHO has proposed a more straightforward classification for treatment purposes, based on cutaneous manifestations and skin smears, which categorizes leprosy into 1) paucibacillary (PB): ≤5 skin plaques and negative smears and 2) multibacillary (MB): ≥6 skin plaques or positive smears.
Up to 75% of individuals with leprosy have ocular manifestations, and 39.40% have a visual disability. , There are a wide variety of ocular manifestations, such as lagophthalmos, madarosis, corneal ulcers, cataracts, uveitis, and iridocyclitis. It has been proposed that Mycobacterium leprae has a preference for the iris, which is due to its safety from the immune system and systemic treatment. The diagnosis of ocular leprosy is challenging in the absence of characteristic skin lesions because of its diverse ocular manifestations and extensive time for development. We describe the first case of a patient with bilateral cataracts and uveitic glaucoma secondary to tuberculoid leprosy confirmed by histopathological studies in Quito, Ecuador.
2
Case presentation
A 62-year-old female was referred to our clinic by an ophthalmologist, as she was diagnosed with anterior uveitis of unknown etiology and ocular hypertension. During the past two years, the patient had suffered from elevated intraocular pressure (IOP) in both eyes (OU) refractory to topical therapy and bilateral anterior uveitis refractory to corticosteroid therapy. For her IOP, the patient was on a fixed combination of timolol-dorzolamide bid in her left eye (OS) and only timolol bid in her right eye (OD). The previous corticosteroid treatment that the patient followed whenever a crisis reappeared was topical prednisolone acetate every 3 h. This medical regimen was maintained for a year. She was not on systemic medications.
The patient presented to our clinic complaining of conjunctival injection in OU, persistent headache, eyelid irritation, and a decrease in visual acuity in the OS. The physical exam revealed mild ptosis in OU and no other findings. Upon ocular examination, her best-corrected visual acuity was 20/25 in the OD and 20/60 in the OS. IOP was 29 mmHg and 22 mmHg in her OD and OS, respectively.
The slit-lamp examination in OU showed the following findings: erythema and scales at eyelid margins, decreased tear break-up time, cornea with diffuse punctate epitheliopathy, mutton-fat keratic precipitates, symmetric, round and reactive pupils, sparse patches of iris atrophy, anterior chamber cells 1+, posterior subcapsular opacities in both eyes denser in the OS, and clear vitreous. Gonioscopy showed open angles in OU and peripheral anterior synechiae (PAS) in the inferior quadrant in the OD and in the superior quadrant in the OS ( Fig. 1 ).
The fundus examination revealed a cup disc ratio of 0.55 × 0.45 in the OD with thinning of the inferior neuroretinal rim and localized loss of the retinal nerve fiber layer (RNFL) in the inferior quadrant; the cup disc ratio in the OS was 0.4 × 0.3. The macula, blood vessels, and peripheral retina were unremarkable as well as fluorescein angiography of the retina in OU. The visual field showed a mild superior arcuate defect and a glaucoma hemifield test (GHT) “outside normal limits” in the OD. The GHT in the OS showed “general depression of sensitivity.” The RNFL deviation map in the optical coherence tomography (OCT) showed a loss of nerve fibers in the inferior quadrant in the OD and no abnormal OS results. The average RNFL thickness was within normal parameters OU; however, RNFL symmetry was 71%. The deviation map in the macular ganglion cell analysis revealed mild thinning in the temporal quadrant in the OD and no abnormal findings in the OS. The average ganglion cell layer (GCL) plus inner plexiform layer (IPL) thickness was in the normal range in OU. The macular thickness OCT showed a central thickness of 241 μm in the OD and 247 μm in the OS. Specular microscopy indicated a cell density of 2387 cells/mm 3 in the OD and 2625 cells/mm 3 in the OS.
The patient was diagnosed with anterior uveitis and cataracts in OU, uveitic glaucoma in the OD, and ocular hypertension in the OS. Given the presenting chronic granulomatous uveitis of unknown etiology, the respective questionnaire and evaluation were performed by internal medicine and rheumatology. This multidisciplinary team ordered laboratory tests to assess immunological and infectious profiles ( Table 1 ). The serological antibodies for toxoplasmosis, rubella, syphilis, tuberculosis, and cytomegalovirus (CMV) showed no active infection. Aqueous tap was also performed to study different infectious etiologies with DNA-PCR. CMV, HSV-1, HSV-2, and Toxoplasma gondii had undetectable levels, meaning a negative result. A smear and culture of the aqueous humor were not done due to insufficient sample quantity; therefore, the DNA-PCR tests were prioritized. The immunological tests were negative for rheumatoid factor (RF) and antinuclear antibodies (ANAs). Anti-neutrophil cytoplasmic antibodies (c-ANCA and p-ANCA) were positive. Because of these results, the patient underwent a complete evaluation and imaging workup that did not reveal a conclusive diagnosis.
| Result | Reference values | |
|---|---|---|
| VDRL | ||
| Not reactive | ||
| Toxoplasma gondii | ||
| IgG (U/mL) | 17.70 | Reactive: ≥ 6.5 Not reactive: < 6.5 |
| IgM (U/mL) | <0.9 | Reactive: > 1.1 Not reactive: < 0.9 Undetermined: 0.9–1.1 |
| Rubella | ||
| IgG (U/mL) | 15.20 | Reactive: > 10 Undetermined: 5–9 Not reactive: < 5 |
| IgM (U/mL) | <0.9 | Reactive: > 1.1 Undetermined: 0.9–1.1 Not reactive: < 0.9 |
| Mycobacterium tuberculosis | ||
| PPD (mm) | <5 | Positive: ≥ 10 Negative: < 10 |
| Cytomegalovirus | ||
| IgG (U/mL) | 8.82 | Reactive: > 1.1 Undetermined: 0.9–1.1 Not reactive: < 0.9 |
| IgM (U/mL) | 0.185 | Reactive: > 1.0 Undetermined: 0.7–1.0 Not reactive: < 0.7 |
| ANA | ||
| 0.7 | Positive: > 1.2 Undetermined: 1.0–1.2 Not reactive: < 1.0 | |
| ANCA | ||
| 6.1 | Positive: >1.0 Negative: < 1.0 | |
| Rheumatoid factor | ||
| (U/mL) | 32.6 | Positive: > 60 Negative: < 60 |
| Aqueous Humor PCR-DNA | ||
| Cytomegalovirus | Not detectable | |
| HSV-1 and HSV-2 | Not detectable | |
| Toxoplasma gondii | Not detectable | |
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