Cases reported "Xerophthalmia"

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1/12. Tear film and ocular surface abnormalities after eyelid tattooing.

    A 45-year-old woman who underwent eyelid tattooing 20 years earlier presented with decreased left vision and eye discomfort. Her ocular history revealed an uneventful LASIK procedure 4 years previously with epithelial problems of the flap followed by S. aureus keratitis. Resultant corneal opacity necessitated a deep lamellar keratoplasty performed 9 months prior to admission followed by frequent epithelial problems. At the time of admission, her left eye had a corneal epithelial defect and both lower eyelid margins revealed subepidermal pigmentation and keratinization. Her initial examination revealed tear instability with increased ocular surface staining scores and advanced tear film lipid layer abnormality in both eyes. Meibography showed bilateral total meibomian gland dropout. Treatment with autologous serum eye drops resulted in full epithelialization. Meibomian gland disease-specific therapy did not result in any change in breakup time, vital staining scores, tear film lipid layer interferometry grades, or glandular dropout state.
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2/12. Optic neuropathy and central nervous system disease associated with primary sjogren's syndrome.

    Three cases of optic neuropathy associated with primary sjogren's syndrome are reported. All three patients had clinical manifestations of primary sjogren's syndrome, although two of the patients did not report sicca symptoms at initial examination. Two patients had focal neurologic signs in addition to optic neuropathy. The differentiation of this syndrome of optic neuropathy, focal neurologic signs, and sjogren's syndrome from multiple sclerosis and antiphospholipid antibody syndrome is important for reasons of treatment and prognosis. This diagnostic differentiation was facilitated by positive tests for xerophthalmia and findings of positive minor salivary gland biopsy. High titers of antinuclear antibody, anti-SSA(Ro), and anti-SSB(La), and the absence of antiphospholipid antibodies provided additional help in the differential diagnosis. In 5 years of observation, none of the patients developed symptoms of multiple sclerosis or additional connective tissue disorders.
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3/12. Subacute aseptic meningitis as neurological manifestation of primary sjogren's syndrome.

    sjogren's syndrome (SS) is an autoimmune disease characterized by inflammatory infiltration and secondary chronic dysfunction of exocrine glands. Systemic (extraglandular) manifestations of the disease occur in one-third of the patients, including a wide spectrum of peripheral and central neurological disorders. We report a case of subacute afebrile aseptic meningitis (AM) as neurological manifestation of primary SS. The neurological symptomatology presented gradual onset and progression, including diplopia, mild headache, pain and stiffness of the neck. The clinical examination pointed out xerostomia and xerophthalmia. diagnosis of SS was confirmed by Schirmer's tear test and histopathology of the labial salivary glands. The neurological involvement was highlighted by gadolinium-enhanced magnetic resonance imaging of the brain which displayed an increased diffuse leptomeningeal enhancement. cerebrospinal fluid (CSF) analysis showed moderate pleocytosis with prevalence of polymorphonuclear leukocytes and increased protein level but no evidence of Ig intrathecal synthesis. A cycle of intravenous steroid therapy led to a complete disappearance of the neurological symptomatology and to normalization of the CSF inflammatory pattern. Given the unusual presentation of this case of AM, which resembled the characteristics of a chronic meningitis rather than those of an acute form, in patients affected by SS we must stress the importance of cephalic symptoms such as headaches and cervical stiffness (even if mild and without fever) as possible signs of central neurological involvement of the disease.
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4/12. diagnosis of systemic sarcoidosis prompted by orofacial manifestations: a review of the literature.

    BACKGROUND: sarcoidosis is a multifactorial systemic inflammatory disorder of unknown origin characterized by many potential signs and symptoms, as well as by the presence of noncaseating granulomas in the organs involved. sarcoidosis also may manifest in the oral and maxillofacial region. CASE DESCRIPTION: The authors describe a patient with xerostomia, dysgeusia, oral burning, xerophthalmia and bilateral parotid enlargement. She was diagnosed as having systemic sarcoidosis on the basis of the histologic findings of a biopsy of the labial minor salivary gland, as well as subsequent diagnostic evalutons. CONCLUSION AND CLINICAL IMPLICATIONS: Enlargement of major salivary glands may be the first sign of sarcoidosis in a patient with few other symptoms or clinical findings suggestive of the disease. This case emphasizes the importance of including sarcoidosis in the differential diagnosis of bilateral parotid swelling associated with xerostomia. It also highlights the dentist's potential role in the diagnosis and dental treatment of patients with systemic sarcoidosis.
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5/12. Chronic bilateral dacryo-adenitis in identical twins: a possible incomplete form of Sjogren syndrome.

    We report an unusual case of chronic bilateral dacryo-adenitis in 10-year-old identical twin sisters. Both girls presented with bilateral lacrimal gland enlargement and developed moderate xerophthalmia and keratitis. Both the lacrimal and minor salivary gland biopsies showed a non-granulomatous inflammatory infiltration of mono-nuclear cells. All granulomatous diseases and neoplasms could therefore be ruled out and only Sjogren syndrome and very few other forms of chronic dacryo-adenitis remained as possible diagnoses. Both patients and their parents were evaluated for auto-antibodies. Very low titres of smooth muscle antibodies were found in one, antinuclear antibodies in two and anti-dsDNA antibodies in all four members of the family. Even though the titres of antinuclear and anti-dsDNA antibodies increased in one of the sisters, both patients did not develop any sign or symptom of a systemic connective tissue disease. During the 6 years' follow up, both patients showed persistent tarsal gland enlargement but no other symptoms apart from a moderate xerophthalmia and occasional mild keratitis.
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6/12. Sjogren-like pluriglandular exocrine insufficiency after drug-induced toxic epidermal necrolysis.

    We present the case of a patient that progressively developed xerophthalmia, xerostomia, cutaneous xerosis and exocrine pancreatic insufficiency 3 months after metamizole-induced toxic epidermal necrolysis. Though the association of sjogren's syndrome and exocrine pancreatic impairment is well established, the Sjogren-like syndrome after drug-induced toxic epidermal necrolysis in association with such a wide exocrine glandular insufficiency has not been previously described, to our knowledge.
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7/12. A diffuse infiltrative CD8 lymphocytosis syndrome in human immunodeficiency virus (hiv) infection: a host immune response associated with HLA-DR5.

    STUDY OBJECTIVE: To describe the clinical, immunologic, and immunogenetic features of a diffuse infiltrative lymphocytic disorder resembling Sjogren syndrome in persons infected with human immunodeficiency virus (hiv). DESIGN: Clinical case study. SETTING: University-affiliated hospitals and outpatient clinics. patients: Consecutive sample of 17 patients. MEASUREMENTS AND MAIN RESULTS: All of the 17 patients had bilateral parotid gland enlargement; 14 had xerostomia and 6 had xerophthalmia. Of the 17 patients, 14 had generalized lymphadenopathy, 10 had histologically proved lymphocytic interstitial pneumonitis, 4 had neurologic involvement, and 3 had lymphocytic infiltration of the gastrointestinal tract. gallium scanning in all of 11 tested patients showed abnormal salivary gland uptake. Minor salivary gland biopsies showed more than 2 lymphocytic foci per 4 mm2 tissue in all of 11 tested patients, the infiltrate consisting predominantly of CD8 cells. Fifteen patients had circulating CD8 lymphocytosis; the principal phenotype of these cells was CD8 CD29 . Rheumatoid factor and antinuclear antibodies were infrequent, and none of the patients had anti-Ro/SS-A or anti-La/SS-B antibodies. HLA-DR5 was significantly more frequent in the black patients (10 of 12) compared with controls (13 of 45). Only one patient developed an opportunistic infection during 544 patient-months of study, and none has died of AIDS. CONCLUSIONS: A distinct syndrome primarily characterized by parotid gland enlargement, sicca symptoms, and pulmonary involvement occurs in hiv infection. This disorder is associated with CD8 lymphocytosis and the presence of HLA-DR5, and appears to be a genetically determined host immune response to hiv.
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8/12. Microvascular submandibular gland transfer; an alternative approach for total xerophthalmia.

    A case of total xerophthalmia is reported in which microvascular transfer of the submandibular gland relieved the dryness with negligible side-effects.
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9/12. Sicca symptom in a patient with hemochromatosis: minor salivary gland biopsy for differential diagnosis.

    This paper reports a case of hemochromatosis with sicca symptom. The patient was a 59-year-old female who had been received a series of intravenous iron injections and blood transfusions because of anemia owing to side-effects of a chemotherapeutic agent. She complained of dry mouth and dry eyes in addition to symptoms of hemochromatosis. The histological appearance of the labial salivary gland, with heavy deposition of iron in acinar and duct epithelial cells and absence of focal lymphoid cell infiltration, did not support a diagnosis of sjogren's syndrome but suggested functional damage of the salivary glands related to iron. The patient was given desferrioxamine, and clinical symptoms improved; re-examined labial salivary gland biopsy showed no iron deposition in any parenchymal cells after the treatment.
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10/12. keratoconjunctivitis sicca following excision of a dermolipoma of the lacrimal gland.

    A 20-year-old woman with subconjunctival mass of the superotemporal region of the globe was operated upon. The mass was found to be a dermolipoma of the lacrimal gland. The removal of the mass caused severe keratoconjunctivitis sicca, which could only be helped with frequent applications of artificial tears.
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