Keratoconus Associated with Hyperimmunoglobulin E Syndrome

Purpose To report a case of keratoconus in a patient with hyper-IgE syndrome. Methods A case report of a 28-year-old man with hyperimmunoglobulin E syndrome (HIES) who presented with chronic eczematous dermatitis of the eyelids and severe eye itching. Results Best corrected visual acuity was 20/25 in the right eye and 20/30 in the left eye. There was a scissoring reflex in both eyes with retinoscopy. Biomicroscopy of the cornea was normal, but corneal topography showed bilateral keratoconus. Conclusions It is difficult to establish a direct correlation between keratoconus and HIES due to the rarity of the latter disorder. It is, however, important to consider this association and obtain a corneal topography to rule out corneal ectasia in patients with HIES.

[1]  M. Erlewyn-Lajeunesse.  Hyperimmunoglobulin‐E syndrome with recurrent infection: A review of current opinion and treatment , 2000, Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology.

[2]  H. Ochs,et al.  Defect in neutrophil granulocyte chemotaxis in Job's syndrome of recurrent "cold" staphylococcal abscesses. , 1974, Lancet.

[3]  S. Duman,et al.  Bilateral Keratoconus Associated with Hashimoto's Disease, Alopecia Areata and Atopic Keratoconjunctivitis , 1999, European journal of ophthalmology.

[4]  A. Pherwani,et al.  Hyperimmunoglobulin E syndrome. , 1994, Indian pediatrics.

[5]  R. Geha,et al.  Regulation of immunoglobulin (Ig)E synthesis in the hyper-IgE syndrome. , 1990, The Journal of clinical investigation.

[6]  W. Hodge,et al.  Atopy and keratoconus: a multivariate analysis , 2000, The British journal of ophthalmology.

[7]  J. Krachmer,et al.  Keratoconus and related noninflammatory corneal thinning disorders. , 1984, Survey of ophthalmology.

[8]  R. Patterson,et al.  Massive polyclonal hyperimmunoglobulinemia E, eosinophilia and increased IgE-bearing lymphocytes. , 1975, The American journal of medicine.

[9]  A. Edwards,et al.  Comparison between Near Infrared Spectroscopy and 133Xenon Clearance for Estimation of Cerebral Blood Flow in Critically Ill Preterm Infants , 1993, Pediatric Research.

[10]  E. S. Bereston,et al.  KERATOCONUS ASSOCIATED WITH ATOPIC DERMATITIS: REPORT OF TWO CASES , 1942 .

[11]  O. Çekiç,et al.  Incidence of keratoconus in subjects with vernal keratoconjunctivitis: a videokeratographic study. , 2001, Ophthalmology.

[12]  D L Easty,et al.  Association between keratoconus and atopy. , 1989, The British journal of ophthalmology.

[13]  M. Galin,et al.  Atopy and keratoconus. , 1958, American journal of ophthalmology.

[14]  D. Leung.  Mechanisms Controlling the Human Immunoglobulin E Response: New Directions in the Therapy of Allergic Diseases , 1993, Pediatric Research.

[15]  P. Copeman.  Eczema and keratoconus. , 1965, British medical journal.

[16]  Madnani Na,et al.  Hyperimmunoglobulin E syndrome. , 2001 .

[17]  R. Buckley,et al.  Extreme hyperimmunoglobulinemia E and undue susceptibility to infection. , 1972, Pediatrics.

[18]  Y. Miyamoto,et al.  Case report: a hyperimmunoglobulin E syndrome with serum inhibitor against immune functions. , 1981, Annals of allergy.

[19]  J. Gallin.  The Hyperimmunoglobulin E Recurrent‐Infection (Job's) Syndrome: A REVIEW OF THE NIH EXPERIENCE AND THE LITERATURE , 1983, Medicine.

[20]  H. L. Bair,et al.  Occurrence of cataracts and keratoconus with atopic dermatitis. , 1955, A.M.A. archives of dermatology.