1/9. 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.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |
2/9. Successful treatment of a patient with primary sjogren's syndrome with Rituximab.We report the course of a 55-year-old woman, the first patient with primary sjogren's syndrome and distal renal tubular acidosis but without lymphoma to be treated with B-cell depletion using Rituximab. Rapidly after B-cell depletion, remarkable improvement in xerostomia occurred, while serological findings and tubular acidosis have been unchanged. In labial salivary gland biopsy, lymphocyte infiltration and particularly CD20-positive cells decreased strikingly. aquaporin 1 (AQP-1) expression in myoepithelial cells was very low before treatment and increased noticeably. Apical AQP-5 in acinus cells likewise increased following Rituximab. In contrast, basolateral NKCC1 was expressed at unchanged intensity before and following Rituximab. The improvement has been sustained and still is most gratifying 10 months after treatment. B-cell depletion may be effective treatment in sjogren's syndrome. Likewise, it may now be possible to separate the immunologic phenomena in sjogren's syndrome from the consequences of prolonged hyposalivation when studying the pathophysiology of xerostomia.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |
3/9. 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.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |
4/9. sjogren's syndrome (sicca complex).A case of sicca complex sjogren's syndrome in a 41-year-old woman is presented. Symptoms related to xerophthalmia and xerostomia were severe but, ironically, served to confuse the clinical impressions of medical personnel managing this patient, resulting in a diagnostic dilemma. biopsy of a labial minor salivary gland followed by histopathologic examination of the tissue provided invaluable information which, together with the clinical picture, aided in the establishment of the delayed diagnosis.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |
5/9. 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.- - - - - - - - - - ranking = 2keywords = labial (Clic here for more details about this article) |
6/9. xerostomia: evaluation of a symptom with increasing significance.xerostomia is the subjective sensation of oral dryness. Although it is most commonly associated with salivary gland dysfunction, it may also occur with normal gland activity. xerostomia may be an early symptom of several morbid systemic conditions with important implications for the medical and dental management of patients. Oral dryness also has negative effects on an individual's emotional well-being and quality of life. The complaint of xerostomia necessitates a complete evaluation of a patient's general health, salivary gland function, and oral motor and sensory abilities. The salivary gland assessment includes symptom review, analysis of glandular secretions, scintiscanning, and minor labial gland biopsy. No single component is sufficient to adequately diagnose the presence, extent, or cause of salivary dysfunction. Treatment of a dry mouth, to date, is mainly palliative in nature, with the intent of preserving oral structures and functions. Better therapies are essential in the management of xerostomia, whatever the cause. The importance of xerostomia as a symptom is increasingly recognized in medicine and dentistry. The dentist is commonly the first health professional to hear this complaint and may be critical in directing a full and appropriate evaluation.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |
7/9. A case of sicca syndrome due to primary amyloidosis.We report the rare occurrence of sicca syndrome associated with primary amyloidosis. A 63-year-old man with apparent keratoconjunctivitis sicca and xerostomia died of the cardiac failure. Neither sialography nor labial gland biopsy revealed findings compatible with sjogren's syndrome. Macroglobulinemia and a positive Bence-Jones protein were noted in urine testing. Amyloid deposition was demonstrated by congo red staining on biopsied rectal tissues, and by typical birefringence under polarized light microscopy. At autopsy, the amyloid deposition was identified histochemically in the submandibular gland and the minor salivary glands of oral cavity.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |
8/9. sjogren's syndrome in the adolescent. Report of four cases.We report three cases of sjogren's syndrome and a case with a probable diagnosis of sjogren's syndrome at the ages of 16 (2 cases), 17, and 18 years. Two patients were affected by systemic lupus erythematosus. All four patients showed periductal lymphocytic infiltration in the labial glands. Two of the three patients underwent parotid sialography that revealed punctate or globular sialectasis. A decrease in stimulated parotid flow rate was noted in three of the cases compared with age-matched healthy females, whereas only one patient was diagnosed as having keratoconjunctivitis sicca.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |
9/9. Etiologies of the sicca syndrome: primary systemic amyloidosis and others.BACKGROUND: The sicca syndrome has been defined as the occurrence of xerostomia and xerophthalmia. sjogren's syndrome is the most common cause of the sicca syndrome; however, these two syndromes are not synonymous and there are many potential etiologies of the sicca syndrome. A less known cause of sicca syndrome is amyloidosis that to date has only been reported in the nondermatology literature. OBSERVATIONS: A 79-year-old man with known amyloidosis presented with persistent xerostomia. He had the classic cutaneous findings of periorbital and "pinch" purpura. A labial biopsy showed diffuse deposition of amorphous eosinophilic material surrounding salivary acini. Apple-green birefringence was noted with congo red staining and the diagnosis was made of amyloidosis in the minor salivary glands causing xerostomia. CONCLUSIONS: The sicca syndrome can be caused by systemic amyloidosis. Because this fact is not in the dermatologic literature, many dermatologists are not aware of this uncommon presentation. The knowledge of the many causes of the sicca syndrome and an understanding of the differences between this and Sjogren's syndromes are important for any dermatologist.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |