Year : 2009 | Volume
: 57 | Issue : 5 | Page : 385--386
Ophthalmic complications including retinal detachment in hyperimmunoglobulinemia E (Job's) syndrome: Case report and review of literature
Vipul Arora, Usha R Kim, Hadi M Khazei, Shivayogi Kusagur
Orbit, Oculoplasty and Oncology Clinic, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India
Usha R Kim
Oculoplasty and Oncology Clinic, Aravind Eye Hospital and Postgradute Institute of Ophthalmology Madurai, Tamil Nadu
Hyperimmunoglobulinemia E (Job«SQ»s) syndrome is characterized by markedly increased levels of immunoglobulin E, recurrent cutaneous and systemic pyogenic infections, atopic dermatitis, and peripheral eosinophilia. Although ocular involvement in Job«SQ»s syndrome is rare, there are reports of keratoconus, staphylococcal chalazia with blepharitis, and endophthalmitis by various authors. We present the first case report of retinal detachment with complicated cataract in Job«SQ»s syndrome.
|How to cite this article:|
Arora V, Kim UR, Khazei HM, Kusagur S. Ophthalmic complications including retinal detachment in hyperimmunoglobulinemia E (Job's) syndrome: Case report and review of literature.Indian J Ophthalmol 2009;57:385-386
|How to cite this URL:|
Arora V, Kim UR, Khazei HM, Kusagur S. Ophthalmic complications including retinal detachment in hyperimmunoglobulinemia E (Job's) syndrome: Case report and review of literature. Indian J Ophthalmol [serial online] 2009 [cited 2020 Sep 25 ];57:385-386
Available from: http://www.ijo.in/text.asp?2009/57/5/385/55076
Hyperimmunoglobulinemia E (HIE) or Job's syndrome is an immunodeficiency that was first described by Davis et al .  The name Job was chosen with reference to the Book of Job 2:7: "So went Satan forth from the presence of the Lord, and smote Job with some boils from the sole of his feet unto his crown." Job's syndrome and Buvkley's syndrome are subsets of the HIE syndrome. Elevated levels of serum immunoglobulin E (IgE) with values reaching >2000 IU (normal  Cell-mediated immunity is often abnormal in patients with HIE; the most common functional defects in host resistance are defective suppressor T cell function and defective neutrophilic chemotaxis.
Patients with HIE syndrome generally present with chronic and intense pruritis associated with widespread eczematous dermatitis which occasionally involve the eyelids. This pruritis is attributed to intradermal mast-cell histamine release triggered by the elevation of IgE.  Staphylococcal skin infection is common and manifests in the form of impetigo, furunculosis, paronychia or cellulitis. The characteristic feature of staphylococcal infection in Job's syndrome is abscess formation without the anticipated degree of erythema and warmth. Pulmonary bacterial pneumonia and emphysema are the most frequent systemic infections and may result in pneumatoceles that become the nidus for further bacterial and fungal infection. The most common infecting organisms are Staphylococcus aureus and Hemophilus influenzae . Job's syndrome has also been associated with scoliosis in 76% of adult patients and hyperextensibility of joints in 68% of patients.  Peripheral blood eosinophilia may be marked, reaching levels of 50-60%. Serum IgE levels are consistently high (more than 10 times the upper limit of normal), even in infancy.
Kim et al .  reported a case of bilateral keratoconous in a 28-year-old male suffering from Job's syndrome. Although this association was of unknown etiology, it is important to consider this in HIE patients. In our patient, corneal topography of both eyes failed to demonstrate any ectasia. Destafeno et al .  reported a case of a 16-year-old female with recurrent chalazia associated with Staphylococcus aureus along with other manifestations of this syndrome. Frohn et al .  and Orhan et al .  have reported cases of corneal ulceration and perforation in patients with Job's syndrome presumably caused by Staphylococcus aureus despite aggressive systemic and topical antibiotic therapy. Haslett et al .  have reported a case of endogenous Candida albicans endophthalmitis in a 24-year-old female.
This is the first case report of retinal detachment with complicated cataract in HIE or Job's syndrome in a young male. Our patient had no history of trauma or signs of uveitis in the involved eye. We speculate that the etiology and pathogenesis of retinal detachment in case of Job's syndrome closely resembles with that of atopic dermatitis, which is an IgE-mediated hypersensitivity reaction. Atopic dermatitis patients usually have multiple breaks that occur in the peripheral retina and vitreous base which may lead to rhegmatogenous retinal detachment and giant retinal breaks.  The etiology for these breaks is attributed to vigorous rubbing of eyes due to intense itching ,which could also be a probable cause of retinal detachment in our case. This detachment is non-inflammatory in nature as photoreceptors' outer segments have been detected in aqueous of eyes of patients with atopic dermatitis with retinal detachment. 
Management in our case was only observation as patient had longstanding closed funnel retinal detachment with hypotony of the globe. Patients with Job's syndrome should be carefully evaluated for detailed posterior segment examination and in cases where media opacity is present, B-mode ultrasonography should be done. This aids in early detection and treatment of retinal detachment, thereby reducing ocular morbidity and preventing visual loss.
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