Mohamed Anjum, Radha Annamalai, Muthayya Muthukumar

Purpose: Blau syndrome is an inflammation that is phenotypically characterised by a triad of polyarthritis, dermatitis and uveitis. Early onset sarcoidosis is its sporadic form.

1. We report the inflammation in a 4 year old boy with recalcitrant granulomatous anterior uveitis and cystoid macular oedema.
2. We give our experience on treatment, visual rehabilitation and prognosis.

Method: A complete ophthalmic evaluation was performed using a slit lamp microscope and fundus examination was done using a 78D and 20D lenses. Laboratory investigations such as complete blood count, Mantoux test, X ray chest, PCR, real time PCR, ELISA, IgG, IgM, IgE, ACE, serum lysozyme, Quantiferon TB Gold were done based on suspicion.

Results: Uveitis was severe,bilateral at presentation with mutton fat keratic precipitates, iris nodules, anterior chamber cells 3+, flare grade 3 and posterior synechiae. Fundus examination showed disc pallor and cystoid macular oedema. The child did not respond to corticosteroids and vision worsened to 6/60, N36 in both eyes. Based on investigations, a combination of tablet prednisolone 1mg/kg body weight, methotrexate 7.5mg weekly with folic acid 5mg and intravenous injection infliximab was given for 2 consecutive months. Vision improved to 6/9, N6 in both eyes.

Conclusion:This case is reported for its rarity.Uveitis known to occur after many years of systemic disease had simultaneous onset in our patient. A genetic analysis will be confirmatory. A definite association with glaucoma has been observed. Early treatment with immunosupressive therapy will prevent blinding complications of uveitis in this disease.