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M.H. Vesaluoma, T.M. Tervo, K. Krootila, M. Seppänen; Hyper-IgE Syndrome in Severe Corneal Stromal Herpes Simplex Virus Keratitis . Invest. Ophthalmol. Vis. Sci. 2003;44(13):4763.
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Purpose:Hyper-IgE syndrome (HIE) is a rare primary immunodeficiency with high morbidity and mortality to severe infections, such as pneumonia. We describe a formerly unrecognized association between severe herpes simplex keratitis and hyper-IgE syndrome. Methods:Three atopic patients were referred to a corneal specialist due to severe corneal stromal inflammation/infection. They were also examined by a specialist in infectious diseases. Blood samples were collected for immunological studies. Herpes simplex virus (HSV) was microbiologically verified in all patients. Two patients had a corneal transplant. One of them presented with rejection problems associated with recurrent HSV, and the other one had altogether 3 re-grafts (the first deep lamellar keratoplasty resulting in a primary graft failure was done due to keratoconus). HIE diagnosis was based on the scoring system by Grimbacher et al. (Am J Hum Genet 1999;65:735). Results:The clinical scores were >30 (>15 demanded for the diagnosis), and serum IgE levels were between 1862 and 3683 (normal range 0-110). All our patients were diagnosed as suffering from hyper-IgE syndrome. Atypically, the patients reported no history of pulmonary infections. Typical staphylococcal skin infections, severe eczema, facial features (asymmetric face, high palate) and delayed shedding of the primary teet were, however, reported. The patients may thus represent a formerly unrecognized subgroup of HIE. Conclusions: Patients with severe corneal problems due to herpes simplex virus warrant a systemic examination for hyper-IgE syndrome, as long term antiviral, antibacterial or antifungal medication may reduce overall morbidity and mortality in these patients. HIE patients receiving immunosuppressive treatments should be carefully monitored.
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