Vision Threat in Hyper IgE Syndrome: A Case Report
Hyper IgE Syndrome (HIES) is a rare primary immunodeficiency characterized by elevated IgE levels and recurrent infections. Ocular involvement in HIES is uncommon, with reported cases of various eye conditions such as conjunctivitis and retinal detachment. A 17-year-old male with HIES presented with decreased vision in both eyes, diagnosed with retinal vasculitis and complicated cataract. Treatment with panretinal photocoagulation resulted in maintained vision. Fundoscopic examination revealed intraretinal hemorrhage and macular changes. The case highlights the importance of recognizing ocular manifestations in HIES for timely intervention.
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Manoj Mathur E-Poster [Title of the E-Poster] [Presenting Author s Name] 9thAnnual State Ophthalmological Conference TOSCON 2024 12thto 14thJuly, 2024 at Katriya Hotel & Towers, Hyderabad A THREAT TO AN VISION IN HYPER IgE SYNDROME A CASE REPORT PRESENTER - DR.KEERTHANA. J, OPHTHALMOLOGY RESIDENT, KRISHNA INSTITUTE OF MEDICAL SCIENCES, SECUNDERABAD. MODERATOR - DR.B.PRANATHI, SENIOR CONSULTANT, KRISHNA INSTITUTE OF MEDICAL SCIENCES, SECUNDERABAD.
9th Annual State Ophthalmological Conference TOSCON 2024 12th to 14th July, 2024 at Katriya Hotel & Towers, Hyderabad INTRODUCTION- Hyper IgE Syndrome (HIES) was first described by Deiwis, Schuller and Wedgewood in 1966. Hyper-Ig E syndrome(job s syndrome) is a rare, primary immunodeficiency distinguished by the clinical triad of atopic dermatitis, recurrent skin staphylococcal infections, and recurrent pulmonary infections and characterized by elevated Ig E levels with an early onset in primary childhood. Ocular involvement in job's syndrome is rare. Although rare, there are few reports of conjunctivitis, keratitis, corneal perforation, xanthelasma, eyelid nodules, chalazion, strabismus, retinal detachment, cataracts, keratoconus, retinal vasculitis.
9th Annual State Ophthalmological Conference TOSCON 2024 12th to 14th July, 2024 at Katriya Hotel & Towers, Hyderabad CASE REPORT - 17yr old male, complaint of decrease in vision in BE since 3 months. k/c/o Hyper Ig E syndrome RIGHT EYE LEFT EYE UNAIDED VISION CF-3 MTRS CF-3 MTRS BCVA 6/18 6/18 ANTERIOR SEGMENT WNL WNL LENS NS-1 ,CORTICAL NS-1,CORTICAL COLOUR VISION On Dilated fundus examination, peripheral intraretinal hemorrhage, areas of macular whitening, perivascular infiltration seen in both eyes. Based on ocular findings and systemic manifestations, the patient was diagnosed as a case of retinal vasculitis and complicated cataract associated with HIES. Panretinal photocoagulation (PRP) laser treatment was performed in both eyes and on follow up,vision was maintained. 17/17 17/17
9th Annual State Ophthalmological Conference TOSCON 2024 12th to 14th July, 2024 at Katriya Hotel & Towers, Hyderabad MATERIALS AND METHOD- k/c/o hyper Ig E syndrome- DOCK 8 mutation positive. Fundoscopic examination revealed intraretinal hemorrhage, areas of macular whitening, perivascular infiltration. Macular optical coherence tomography of the macula showed epiretinal membrane,inner and outer retinal layers distortion with some atrophic areas. [Fundus photos showing retnal whitening,peri-vascular sheathing.]
9th Annual State Ophthalmological Conference TOSCON 2024 12th to 14th July, 2024 at Katriya Hotel & Towers, Hyderabad Fundus photos- showing peripheral vascular sheathing around the retinal arteries and veins in both eyes. OCT MACULA of both eyes
9th Annual State Ophthalmological Conference TOSCON 2024 12th to 14th July, 2024 at Katriya Hotel & Towers, Hyderabad DISCUSSION- CONCLUSION- The prevalence of vascular abnormalities in HIES is unknown. In patients with HIES, vascular abnormalities of the brain, vein, skin, lung, aorta, heart, and foot have been reported. Retinal vasculitis has been described in common variable immunodeficiency . The cause in that entity and in our case could be related to autoimmunity or to the occlusion from the high level of IgE as it can induce synthesis of the leukotrienes, chemokines, and cytokines leading to aggregation of the leukocytes and eosinophils. Patients with Job's syndrome should be carefully evaluated for detailed ophthalmic examination. Early diagnosis and intervention increase patient s quality of life, improves visual prognosis and prevents blindness.
