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Ocular Manifestations of Behcet's Disease 

 Behcbehcet's ocular manifestationset's disease is a systemic inflammatory vascular disorder characterized by recurrent oral and genital ulcers, eye lesion, arthritis and skin lesions. We report a case of Behcet's disease with ocular manifestation in an 8 year old boy. 

 Key words: Behcet's disease, Ocular manifestation. 

 Behcet's disease is a systemic inflammatory vascular disorder characterized by recurrent oral and genital ulcers, uveitis arthritis and skin lesions. It has a worldwide distribution with clustering among populations having a high prevalence of HLAB5. There are only a few reports of Behcet's disease with ocular manifestation in children from India.

Case Report An 8-year-old student from West Bengal presented with a history of fever, recurrent oral and genital ulcerations, skin pustules and joint swelling for 4 months. There was a history of bilateral parotid swelling at the onset of illness. He had been hospitalized elsewhere for 2 months for the above symptoms and had received antibiotics, IV Immunoglobulin, two blood transfusions, and was on nasogastric feeds.

 On examination child was febrile (102ºF) toxic and undernourished. Weight was 17.7 kg. He had bilateral enlarged tender submandibular and upper deep cervical lymphnodes. Ocular examination revealed nodules over the bulbar conjunctivae, conjunctival congestion and bilateral purulent eye discharge (Fig.1). Fundus examination was normal. He had multiple oral ulcers, few covered with slough and few covered with crusts, seen on the tongue and upper and lower lips. There was a pustule on the left angle of mouth. Multiple pustular lesions of size 1-2 cm were present over the face, arms, both knees, dorsum of the feet and IV puncture sites; some had healed and left scars. Perianal and periurethral areas were normal. Tender swelling of right knee joint with minimal effusion was present. The examination of other systems was unremarkable. Hemoglobin was 10.6 g/dL. Total white cell count was 21,400/mm3 with 90% neutrophils. Platelets count was 5,89,000/mm3. Liver function test, S. creatinine, urea, calcium and electrolytes were normal. ANA, Rheumatoid factor and LE cell preparation were negative. Serum complement was 80%. VDRL was nonreactive; CRP was positive 94.6mg/L. HIV serology and HBs Ag were negative. Smear & culture from the buccal ulcer was negative for Herpes simplex virus. Pus swab culture from ulcer in the right foot grew Pseudomonas aeruginosa. CSF analysis was normal.   

HLA typing was positive for B52(5). Chest X-ray was normal. Ultrasonography of abdomen and upper gastrointestinal endoscopy were normal. Slit lamp examination of his eyes revealed no evidence of uveitis. Bone marrow smear showed hypercellular marrow with mild increase in reactivity and biopsy showed hypocellular marrow with myeloid hyperplasia. Skin biopsy from right leg showed intra-epithelial vesicle with neutrophilic infiltration, deep dermal granulomatous inflammation, moderate perivascular chronic inflammation and telangiectasia in the dermis. Pathergy test was positive.

 At admission the differential diagnoses considered were connective tissue disorder, immunodeficiency syndrome, lympho-proliferative disorder, and disseminated HSV infection. He was initially treated with IV antibiotics and intravenous acyclovir.

Later the possibility of Behcet's disease was considered in view of the oral ulceration, typical skin (vesicles, pustules and pyoderma gangrenosum) and eye lesions, genital ulceration and positive pathergy skin test fulfilling the recommended diagnostic criteria(1). HLA typing was positive for HLA B5 and skin biopsy was suggestive of Behcet's disease.

He was started on oral prednisolone (60 mg/m2/day) and local eye application of Fluromethonolone drops. He became afebrile from the 2nd day of starting steroids; skin and ocular lesions also improved. Right knee joint swelling was treated with skin traction and it resolved within one week. He was discharged on oral feeds. 

At follow-up a month later his skin lesions had improved and the steroid dose was tapered and stopped after 4 months. Two weeks later he presented with recurrence of eye lesions and oral ulcerations. He was restarted on oral steroids and continued for a year and stopped . At his last visit one and half years after the diagnosis and one month after stopping steroids, he was well with no skin or oral lesions. The parents were counselled regarding the prognosis and the risk of recurrence of manifestations of the disease.   

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