Search

Background: Behcet's disease (BD) is a chronic, recurrent, multi-system inflammatory disorder involving mucocutaneous (MC), ocular, intestinal, articular, vascular, urogenital and neurologic systems. BD occurs with a high prevalence in the Mediterranean population. There is scarcity of clinical data on BD from India with only three case series in the last two decades., Aims: To study demographic profile, clinical manifestations and treatment outcome of patients with BD presenting to the dermatologic clinic in a tertiary hospital in north India., Methods: Prospective analysis of all patients diagnosed to have BD between 1997 to 2011., Result: Twenty nine patients were diagnosed to have BD. The disease had a female preponderance (M:F = 1:3.8) with a mean age of disease onset of 27.4 (range 16-61) years. The prevalence of various MC and systemic manifestations are as follows: oral aphthae (100%), genital aphthae (93.1%), erythema nodosum (62%), papulopustular and acneiform lesions (31%), articular involvement (68.9%), ocular involvement (31%) and gastrointestinal (GI) involvement (3.4%) . Pathergy test positivity was observed in 31%. The treatment comprised of colchicine (16/29 patients), dapsone (7/29), dapsone with pentoxiphylline (3/29), systemic steroid (2/29), systemic steroid with methotrexate (1/29). Colchicine was effective and well tolerated in all patients., Conclusion: The disease occurs in a much milder form in India and is primarily mucocutaneous and arthritic. A high index of suspicion in patients with MC lesions may result in early diagnosis, management and prevention of complications of BD. We suggest colchicine as an effective and safe therapeutic option for MC and joint involvement.

Epidermal nevus syndrome (ENS) is a rare disease, the pathogenesis of which is largely elusive. We, hereby, report an exclusive case of a 20-year-old man with verrucous ENS presented with dark colored papules and plaques along the Blaschko's lines present over the head and neck area along with fleshy growth in both eyes since birth. Limb length discrepancy and kyphoscoliosis were remarkable. Skin biopsy was compatible with verrucous epidermal nevus while the biopsy of the ocular lesion confirmed complex choristoma. MRI brain revealed calcification in the right temporal lobe. Bilateral arachnoid cyst in the middle cranial fossa, scleral osteoma in the posterior part of the right eyeball, and deformed calvarium were evident on CECT skull and orbit. The present illustration emphasizes the importance of a punctilious work up of the case.

Congenital ectropion uveae (CEU) is a rare, non progressive anomaly characterised by the presence of iris pigment epithelium on the anterior surface and is frequently associated with anterior iris insertion, dysgenesis of the drainage angle and glaucoma. This paper describes an unusual case of bilateral congenital ectropion uveae with iris coloboma and telecanthus. The anterior chamber angle was normal and there was no evidence of glaucoma. To the best of our knowledge, this association has not been reported previously., (Copyright © 2011 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.)

Cutaneous tuberculosis may be associated with concurrent systemic foci in the body such as lung, lymph node, bone or CNS. Phlyctenular keratoconjunctivitis (PKC) is a manifestation of immunological response to a variety of antigens in the eye, tubercular focus (evident or occult) being the commonest in India. Reports in the existing literature have shown lungs and lymph nodes to be the predominant underlying focus associated with PKC, whereas cutaneous tuberculosis has seldom been found in this situation. We report this forgotten association in two children with cutaneous tuberculosis, one each with lupus vulgaris and scrofuloderma, who also had PKC. Interestingly, one of the cases also had simultaneous lichen scrofulosorum, which is also an immunological response to tubercular antigen and manifests in the skin, thus showing immunological manifestation in two different organ systems along with cutaneous focus of tuberculosis.

Purpose: Serial evaluations of total, basal and emotional tear secretion in full-term normal neonates to determine the time after birth when these parameters attain normal adult values., Method: Both eyes of 102 full-term normal neonates were prospectively evaluated for tear secretions in the Department of Ophthalmology, over a period of one year. Serial recording of the Schirmer I test (total tear secretions), Basal Secretion Test, and Schirmer II test (emotional tears) was done, the first one being within 6 hours of birth. All the tests were repeated in each infant until normal adult values for each test were obtained., Results: The average values of the Schirmer I test, Basal Secretion Test and Schirmer II test at birth were 23.2 (+/- 3.96) mm, 6.2 (+/- 2.15) mm and 19.2 (+/- 4.94) mm, respectively. A statistically significant correlation of birth weight but not of gestational age was found with basal secretions (p = 0.004) as well as with emotional tears (Schirmer II test, p = 0.010). At birth, 98% of infants had total tear secretion, 3.9% had basal tear secretion and 2.9% had emotional tear secretion comparable to normal adult values. All the parameters of tear secretions increased with time, so that 100% of infants had total tear secretion comparable to normal adult values within 12 hours of birth. The basal secretions took three weeks and emotional tears took four weeks to attain normal adult values in all the neonates., Conclusion: Total tear secretions were the earliest to reach normal adult values, followed by basal secretions and, lastly, emotional tears. Reduced basal tears at birth may predispose neonates to corneal drying during prolonged ocular examinations such as indirect ophthalmoscopy and procedures under general anesthesia.

Objective: Improved survival of low birth weight, premature babies have increased the incidence of retinopathy of prematurity. This hospital-based, prospective, study was undertaken to determine its incidence and risk factors in our neonatal unit., Methods: Neonates with gestational age < or = 35 weeks and/or birth weight < or = 1500 gm born over a one-year period were examined by indirect ophthalmoscopy between 2 to 4 weeks after birth, and followed up till retinal vascularisation was complete. Maternal and neonatal risk factors were noted and data analyzed by statistical package SPSS-10.0., Results: Sixty babies were thus examined. The incidence of retinopathy was 21.7% in the cohort, 33.3% in babies < or = 32 weeks gestation and 36.4% in babies weighing < or = 1250 gm. Oxygen (p=0.01), sepsis (p=0.04) and apnoea (p=0.02) were independent risk factors. Retinopathy was significantly more severe in babies with hyaline membrane disease (p=0.02) and lower birth weight (p=0.02). Severe disease was never seen before 6.5 weeks of age., Conclusion: Indirect ophthalmoscopy should be performed at 4 weeks of post natal age in all preterm babies with birth weight < or = 1500 gm, and intensified in the presence of risk factors like oxygen administration, apnoea and septicemia.

One hundred and six consecutive patients (old and newly diagnosed) attending the leprosy clinic at Guru Teg Bahadur Hospital, Delhi, underwent a detailed ophthalmic examination to identify factors associated with the prevalence of sight threatening lesions of leprosy so as to delineate a sub-group of patients who should continue under active ophthalmic surveillance to prevent blindness. All of them were receiving multidrug therapy as recommended by WHO. Demographic data, systemic parameters and ophthalmic lesions attributable to the disease were endorsed on to a proforma. The data were analysed by chi-square test, discriminant analysis and multivariate regression analysis to isolate factors that could be associated with sight threatening lesions of leprosy. In 106 leprosy patients 212 eyes were examined; sight threatening lesions were found in 37 eyes (17.4%). The comea was involved most commonly (78.3%). The lesions were still present in 15.8% of patients who had completed multidrug therapy. Leprosy related visual impairment was seen in 2.3% eyes and blindness in 0.9% eyes. Factors significantly associated with sight threatening lesions included female gender, multibacillary leprosy, increasing duration of leprosy, thickened corneal nerves, subepithelial punctate corneal opacities and presence of any academic lesion of leprosy. On multivariate and discriminant analysis, female gender, thickened corneal nerves and duration of leprosy > 7 years were significantly associated with sight threatening lesions.

Two cases of linear nevus sebaceus syndrome (LNSS) are described in which ipsilateral facial nevus of Jadassohn was associated with complex ocular choristoma. One patient also had scleral osteomas, a rare occurrence in LNSS.

Corneal affections cause severe ocular morbidity in leprosy. Poor nutrition and low socio-economic status make the eyes prone to repeated secondary infections which makes the pattern of corneal disease in this country different from that reported in western literature. A study of 250 patients shows that leprotic keratopathy has 4 different patterns. Primary leprous keratitis was seen in 56.5% of cases, while secondary leprous keratitis (groups B, C & D) constituted 57.7%. In the latter group the ocular morbidity could be prevented by controlling infection and prevention of concomitant diseases. Cases of lepromatous leprosy showed a consistently higher incidence of different types of corneal involvement than tuberculoid cases.

Argon laser iridotomy was successfully performed in 30 eyes of glaucoma patients. A modified two stage procedure has been described to achieve a successful iridotomy in pigmented eyes. At the chosen site of laser iridotomy, partial thickness burns were applied in the first stage followed by a penetrating burns in the second stage. This minimised the complications associated with laser iridotomy and decreased the incidence of subsequent closure. At the end of 6 months follow up the success rate was 93.3 percent.

Corneal involvement in leprosy is known to be influenced by factors such as lagophthalmos, ectropion, and corneal anesthesia. In the present study conducted on 400 eyes of leprosy patients, observations were made on tear film abnormalities, and their relation to the above-mentioned factors was analyzed. The abnormality of tear film breakup time (BKUT) was found to be clinically more significant (47.2%) than a Schirmer's test alone (24.5%), and this finding was of greater significance in the lepromatous form of leprosy. An abnormal BKUT reflects a decreased mucin secretion by the conjunctival goblet cells. Tear film abnormalities in association with lagophthalmos, corneal anesthesia, concomitant ocular diseases, and environmental factors such as high relative humidity have been identified as factors contributing to corneal involvement in leprosy in India. It is not surprising that with so many mechanisms of corneal involvement the clinical manifestations are diverse, and all forms of pathological changes may contribute to corneal morbidity.

A total of 424 leprosy patients were screened for uveal involvement. Uveal changes were found in 11.2% of these patients. Uveal involvement was more common in patients with greater duration of the disease and in patients on irregular Dapsone therapy. Of the patients with uveal involvement 82% were lepromatous, 16% were borderline and 2% were of tuberculoid leprosy. Uveal changes in form of active iridocyclitis (21.3%), healed iridocyclitis (58.5%), iris atrophy (10.6%), iris pearls (7.4%), small irregular pupil (46.7%), pupil refractory to dilatation (56.3%) were seen. Posterior segment involvement was rare. Lepromatous and borderline leprosy patients with no clinical evidence of uveal involvement had decreased power of accommodation as compared to normal subjects.

The present study analyses the precorneal teat film using schirmer's test and tear film breakup time (BKUT) in 400 eyes of patients with various types of leprosy. An abnormal tear film BKUT (47.2%) was observed to be much more informative in the present series than the study of schirmer's test alone (25.4%). In patients with lagophthalmos an abnormal tear film BKUT (70%) was higher as compared to an abnormal schirmer's test (40%); while in cases of lagophthalmos with ectropion an abnormal schirmer's test was seen less frequently (26.8%) than in cases of lagophthalmos without ectropion (58.3%). This implied an increase in the aqueous content of the tear film in cases of lagophthalmos with ectropion on the basis of an associated chronic conjunctivitis. Lagophthalmos and ectropion have been identified as additional factors contributing to corneal morbidity. There was a statistically significant difference in the tear film abnormality in different types of leprosy, the maximal difference being in the lepromatous variety.