Behcet's disease.

Behcet's disease is a recurrent multisystem vasculitis that can affect any organ or system, but was originally described as orogenital ulcerations and uveitis. Specific criteria have been proposed for diagnosis and identification of affected organs by different national authorities. Behcet's disease is proposed to be due to an antigen/antibody reaction. The antigen can be external (microbial or other) or self-antigen. Self-antigens include HLA-B, S and interphotoreceptor retinoid binding protein, the oral mucosal antigens, and alphatropomycin. The antibody reaction manifests as changes common to any inflammatory process. Circulating immune complexes and neutrophil hyperactivity are present with the expression of adhesion molecules related to disease activity associated with phenotypic and functional aberrations affecting both Th1 and Th2 activity. The results in an increase of interleukin-4, -10, and -1B and interleukin-2 receptors with deficient interleukin-12, interferon-gamma, and expression of cell adhesion CDII/CD18, accompanied by increased B cells secreting IgG. The clinical picture varies according to the organ affected; emphasis is placed on those of diagnostic importance and those affecting morbidity and mortality. The histopathology demonstrates vasculitis with inflammatory cell infiltration, the endothelium expressing ICAM-1 and E-selectin, vascular occlusion with sequences. Treatment includes corticosteroids. Other immunosuppressant and biological agents include anti-tumor necrosis factor, interferon-alpha, Campath-1, and the S antigen. Colchicine and other miscellaneous therapies are included.

Immunopathogenesis of toxoplasmosis.

Toxoplasmosis is a ubiquitous infection affecting 500 million persons around the world, with a range of incidence 12%-90%, increasing with age, education, crowding and sanitary habits. Cats are the definitive host. Infection is primarily congenital but acquired ocular infection has been documented. The review focuses on the immunopathogenesis of toxoplasmosis with emphasis on the eye problem due to its morbidity. The response to infection correlates with both parasitic and retinal antigens. The clinical picture and histopathology is a reflection of the immune response which constitutes early humeral response which presents both systemically and locally primary infection and is elevated with reactivation. This is followed by the cellular response, varying from low grade monocular infilterate a total tissue destruction with response of live parasites especially in immuno-compromised patients. Penetration of the parasite and its enclosure within the parasitophorous vacuole and its eneyst all make its eradicatier impossible. This reflects the variability of antiparasitic therapy that include folate antagonists, macrolides, hydroxy naphthoquindones and fluoroquinolones. Use of corticosteroid should be limited to severe reactions and should be associated with specific therapy.

Sarcoidosis presenting as multilobular limbal corneal nodules.

PURPOSE: To review reported external ocular manifestations of sarcoidosis and to present bilateral, multilobular, nodular, limbal, corneal nodules as being a unique manifestation of the disease. PATIENTS AND METHODS: A 16-year-old Saudi girl presented with bilateral, multilobular, solid, limbal nodules, with a vascular supply from the conjunctival vessel, and associated membraneous conjunctivitis and healed trachoma. The Schirmer's test revealed less than 2 mm in both eyes with tear meniscus less than 2 mm. Biopsy of an associated palpebral conjunctival nodule was performed, in addition to a gallium scan, chest X-ray, and a serum angiotensin-converting enzyme (SACE) level. RESULTS: The culture showed beta-hemolytic streptococci. Gallium scan showed intake by both lacrimal glands. Her chest X-ray results were normal, as was the SACE level. Biopsy of the excised conjunctival nodule disclosed a noncaseating granulomatous reaction with epithelioid and giant cells, and chronic inflammatory cell infiltrate confirming a diagnosis of sarcoidosis. CONCLUSION: A multilobular, nodular, perilimbal mass as a unique manifestation of sarcoidosis is presented. A streptococcal membraneous conjunctivitis and healed trachoma superimposed. A review of sarcoidosis of the external eye is included.

Ocular sarcoidosis in Kuwait with a review of literature.

OBJECTIVE: To study 18 cases of sarcoidosis, the hall mark of which was uveitis. SETTING: Referrals from peripheral eye clinics. PATIENTS: 18 patients with bilateral almost symmetric uveitis, negative Mantoux test and positive gallium scan were enrolled in the study. Preliminary bronchoscopy and bronchial lavage were not done due to social habits. RESULTS: Age ranged between 7-48 years with median 15 and mean 19 years. Although the patients were multinational, yet all of them were residents of Kuwait. A father and son were affected within 8 months period. 78% were strictly ocular, associated pulmonary and salivary gland affection 11% each. Sole anterior uveitis was found in 28%, associated with intermediate uveitis in 55% and with posterior in 16.6% of cases. Clinically detectable dry eyes associated 33% of cases. All our patients developed glaucoma which resolved with treatment of uveitis in 88% of them. Gallium uptake of the eye balls was found in 22%, of the lacrimal glands (panda sign) in 67%, and of the salivary glands or chest 11% each. Positive biopsy was found in 72%, 22% of which was conjunctival. Chest X-ray and SACE were positive in 11%. 61% had hypergammaglobulinaemia and all had negative ANA and RF. CONCLUSIONS: (1) 61% of ocular sarcoidosis presented below sarcoid age. (2) Multinationality together with father's and son's affection indicate a climatic or environmental insult in an already predisposed person. (3) Routine chest X-ray and SACE may not be adequate for diagnosis of ocular sarcoidosis. Gallium should be done in suspected cases. (4) Follow-up for prospects is emphasized.

Congenital hereditary autosomal recessive alacrima.

This report describes two phenotypically normal sisters, each married to a phenotypically normal paternal cousin. One couple had one female and one male child with alacrima; the other couple had a similarly affected girl. The keratitis sicca was manifested by punctate staining of the conjunctiva and blotchy staining of the cornea in the interpalpebral zone with fluorescein and Rose Bengal. Apart from an associated atopy in one of the children, pharmacological testing, biochemical analysis, and clinical examination suggested dysfunction of the lacrimal glands. We propose that the alacrima in this family is inherited in an autosomal recessive fashion.

Conjunctival ulcer in a patient with Crohn's disease.

A 38-year-old woman with documented Crohn's disease (CD) had uniocular nodular episcleritis and a limbal nodule with surrounding infiltrate, which, when healed, left residual pannus. The episcleral nodule was abnormally large (7 x 5 x 3 mm). A localized conjunctival ulceration, a rare manifestation, to our knowledge not previously reported, developed later in the same eye. Impression cytology of the ulcer showed pyknotic conjunctival epithelium and absence of goblet cells, together with the presence of a few plasma cells, lymphocytes, and macrophages. These features are comparable to those seen in the intestinal mucosa of patients with CD. The unilaterality of the ocular infection together with the good response to local therapy suggests that a local antigen-antibody immune complex reaction was involved. We recommend local therapy as a first treatment option.

Congenital alacrima without associated manifestations (AD). An affected father and son.

The authors report a Kuwaiti Arab family in which the father and one of his two sons have severe hypolacrimation with blotchy staining of the cornea and punctate staining of the interpalpebral bulbar conjunctiva by fluorescein and Rose Bengal. Pharmacologic testing together with biochemical analysis and systemic examinations and investigations suggest an isolated dysfunction of lacrimation. The authors' small Arab family differs from the only other recorded pedigree (Irish) in which all five affected members in four generations also had atopy.

Herpetic stromal keratitis in congenital dysgammaglobulinemia. Case report.

The clinical picture and laboratory findings of a case of secondary herpes simplex virus type 2 in a patient with congenital dysgammaglobulinemia who was followed for 26 months is described. Local combined therapy of acyclovir and Decadron (dexamethasone) 0.1% was given for management for 14 months. The clinical and immunopathologic findings of our patient match the results described by other investigators in experimental animals.

Defective vision among Kuwaiti students.

A study, which was intended to find out the main causes of defective vision among Kuwaiti students, yielded the following results: The percentage of defective vision among 139,769 Kuwaiti students is 0.099%. Hereditary and genetic causes are responsible for 75.5% of the cases. Recessive inheritance account for 36.2% Autosomal dominant account for 34.3% Sex linked 9.5% Multifactorial 20.0% Forty-nine cases (of the total number of hereditary and genetic cases), i.e., 46.7% are the product of consanguineous marriages. Inflammatory causes account for 20.9%. The most common cause is neglected cases of muco-purulent and purulent conjunctivitis, becoming corneal ulcers and ensuing complications. This accounts for 62.1% of the total number of inflammatory causes. Traumatic, metabolic, and neoplastic causes account for 3.6%. Comparative data between England and Wales, Australia, Cyprus and Lebanon together with recommendations for improvement are discussed.