Behçet's disease in patients of German and Turkish origin--a comparative study.
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Biomedical subjects
Publications and source records attributed to Manfred Zierhut.
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Dendritic-cell (DC) populations throughout the body have a wide range of features in common, which are associated with their primary function in antigen presentation. The unique immune milieu of the anterior segment of the eye is characterized by a selective DC-dependent inability to develop delayed hypersensitivity responses following antigen invasion into the eye. Recent research papers provide evidence that different maturation stages of DC subsets are detectable at different corneal sites. Thus, the corneal DC, as well as the DC and the macrophages in the iris and ciliary body, have the potential of determining the outcome of immunity or tolerance within this organ.
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BACKGROUND: The keratitis-ichthyosis-deafness (KID) syndrome (Senter Syndrome) and its major criteria erythrokeratodermia, neuro-sensoric deafness and vascularising keratitis were defined in 1981. Several cases have been described since 1915, but up to now no causal therapy of this disease with sporadic occurrence has been found. Clinical experience using systemic Cyclosporin A (CsA) in the dermatological therapy of the KID syndrome differs. Up to now there has been no report on the use of topical CsA eye drops in the therapy of vascularising keratitis. HISTORY AND SIGNS: We report on a case of keratitis, ichthyosis and deafness (KID syndrome) in a 36-year old male. Both eyes were suffering from recurrent corneal ulcers (ulcus serpens corneae). No improvement had been observed on topical or systemic medication (including lubricants, antibiotics, steroids, etc.) during a history of more than two years. THERAPY AND OUTCOME: Topical CsA 2 % (eye drops, 3 times daily) was administered in addition to lubricants. After 3 months the deep corneal neovascularisations were significantly reduced. The corneal ulcer was closed and visual acuity increased from 20/400 (OU) to 20/100 (OD) and 20/200 (OS), respectively. CONCLUSION: A dose-dependent keratinocyte growth inhibition was found in vitro for cyclosporin A using ichthyosis skin samples. The reduction of activated T-lymphocytes and the resulting reduced HLA-Class-2 expression of conjunctiva and cornea as well as the inhibition of activated T-Cells in the lacrimal gland might be responsible. Nevertheless, the effect of topical CsA on the vascularizing keratitis in the KID syndrome is unknown.