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Behcet's disease is an inflammatory systemic disease characterised by four major symptoms such as oral aphthous ulcers, genital ulcers, skin lesions, and intraocular inflammation. 1 Vogt-Koyanagi-Harada (VKH) disease is also associated with panuveitis as an ocular sign, and is accompanied by meningismus, cerebrospinal fluid pleocytosis, auditory findings and integumentary findings. 2 Genetic factors are implicated as one of the pathogenetic signs of both diseases. 3 Some patients have risk alleles such as human leukocyte antigen (HLA)-B51 and DR4 but others do not. We experienced sister cases having the same HLA haplotypes; the elder sister became ill with Behcet's disease and the younger sister was affected with VKH disease.
Case 1
A 54-year-old woman was referred to our hospital complaining of blurred vision in the left eye in 2002. She had had a history of oral aphthous ulcers for 40 years, genital ulcers and erythema nodosum for 15 years. Hypopyon iridocyclitis and retinal vasculitis with subretinal haemorrhages were seen and fluorescein angiography showed diffuse retinal capillary leakage ( fig 1 ). She was diagnosed with Behcet's disease. Her HLA genotype was HLA-B*4002, B*5101, DRB1*0901 and DRB1*1401. Later, she experienced severe intraocular inflammation OU. Ocular attacks were repeated and were resistant to topical corticosteroids including sub-Tenon injection and eyedrops. She was treated with colchicine and ciclosporin. In August 2006, we started anti-tumour necrosis factor α (infliximab) therapy. After the initiation of infliximab, she has had no more ocular attacks.
Case 2
In 2004, a 54-year-old woman visited our clinic complaining of decreased vision OU. Cases 1 and 2 are sisters and case 2 is two years younger than case 1. She had fever for three days and had headache...