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    1444 results match your criteria Oral Manifestations of Systemic Diseases

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    Tuberculosis 'The Great Imitator': A usual disease with unusual presentations.
    Indian J Tuberc 2017 Jan 4;64(1):54-59. Epub 2016 Jul 4.
    Lecturer, Dept. of Pathology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India.
    Background: A number of infectious diseases have been referred to by the phrase 'The Great Imitator', of which the oldest is syphilis; others include Lyme disease, nocardiosis, etc. Tuberculosis has been described as the second great imitator as it can imitate various other disease processes. An awareness of the atypical clinical manifestations of tuberculosis is important, especially in regions where tuberculosis continues to be a major public health problem, such as India. Read More

    Three Cases of Erdheim-Chester Disease with Intraocular Manifestations: Imaging and Histopathology Findings of A Rare Entity.
    Am J Ophthalmol 2017 Jan 30. Epub 2017 Jan 30.
    The Rotterdam Eye Hospital, Het Oogziekenhuis Rotterdam / The Rotterdam Eye Hospital, Postbus 70030, 3000 LM Rotterdam, The Netherlands.
    Purpose: To report intraocular manifestations of ECD with multimodal imaging.

    Design: A retrospective observational case series.

    Methods: This was a multi-center case series of three patients with confirmed tissue diagnosis of ECD that showed intraocular manifestations and were imaged at baseline and follow-up visits. Read More

    Autoimmune Disease Manifestations in the Oral Cavity.
    Surg Pathol Clin 2017 Mar 28;10(1):57-88. Epub 2016 Dec 28.
    Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida, 1395 Center Drive, Gainesville, FL 32610, USA.
    Immune-related disorders of the oral cavity may occur as primary disease process, secondary to systemic disease or neoplasm, or as a reaction to medications and other agents. The entities represented within this group may vary significantly by severity, clinical presentation, microscopic presentation, and special testing results. The selected immune-related conditions of the oral cavity in this article are categorized and presented by their prototypical tissue reaction patterns: vesiculobullous, including acantholytic and subepithelial separation; psoriasiform; spongiotic; and lichenoid reaction patterns. Read More

    Evaluation of olfactory function in Behçet's disease.
    Eur J Rheumatol 2016 Dec 1;3(4):153-156. Epub 2016 Dec 1.
    Division of Rheumatology, Department of Internal Medicine, Ondokuz Mayıs University School of Medicine, Samsun, Turkey.
    Objective: Behçet's disease (BD) is a chronic, relapsing type of vasculitis of unknown etiology and is characterized by oral and urogenital ulcers and ocular inflammation with cutaneous, musculoskeletal, vascular, and nervous system manifestations. Few cases involving the nasal mucosa have been reported in the literature, and the true prevalence of BD remains unknown. Neurological involvement associated with BD might play a more important role in causing olfactory dysfunction than mucosal involvement, but sufficient clinical data are not available on the effect of BD on olfaction in adults. Read More

    [Fabry disease].
    Ann Dermatol Venereol 2017 Feb 16;144(2):137-146. Epub 2017 Jan 16.
    Département de dermatologie, Hôpital Hôtel-Dieu de France, Faculté de médecine, Université Saint-Joseph, Beyrouth, Liban.
    Fabry disease, also known as Anderson-Fabry disease or angiokeratoma corporis diffusum universale, is an X-linked recessive form of sphingolipidosis caused by total or partial deficiency of the lysosomal hydrolase, alpha-galactosidase A. From the youngest age, it results in a gradual ubiquitous build-up of glycosphingolipids that are not degraded by the missing enzyme. Cutaneous, neurological, nephrologic, cardiac, gastrointestinal, ophthalmological, respiratory, cochleovestibular and haematological involvement are responsible for increased mortality and significant impairment of quality of life in subjects affected by the disease. Read More

    Mechanisms and New Strategies for Primary Sjögren's Syndrome.
    Annu Rev Med 2017 Jan;68:331-343
    Department of Physiology, School of Medicine and Joint Academic Rheumatology Program, National and Kapodistrian University of Athens, Athens, Greece; email:
    Primary Sjögren's syndrome (SS) is a common chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, mainly salivary and lacrimal, resulting in oral and ocular dryness, although virtually any organ system can be affected. SS-related systemic manifestations are classified as either related to the presence of periepithelial infiltrates in exocrine and parenchymal organs or resulting from immunocomplex deposition due to B cell hyperactivity with increased risk for B cell lymphoma development. Activation of both innate and adaptive immune pathways contributes to disease pathogenesis, with prominent interferon (IFN) signatures identified in both peripheral blood and affected salivary gland tissues. Read More

    Oral mucous membrane pemphigoid and pemphigus vulgaris-a retrospective two-center cohort study.
    Oral Dis 2017 Jan 13. Epub 2017 Jan 13.
    Division of Oral Medicine and Dentistry, Brigham and Women's Hospital, Boston, MA, USA.
    Objectives: Few studies have compared oral mucous membrane pemphigoid (MMP) and pemphigus vulgaris (PV). Descriptive analysis of oral features, extent of extra-oral involvement, and management outcomes were performed.

    Subjects And Methods: Patients with PV and MMP, the latter with exclusive oral involvement at first presentation, were included. Read More

    ANA-Negative Presentation of SLE in Man with Severe Autoimmune Neutropenia.
    Case Rep Med 2016 19;2016:6853936. Epub 2016 Dec 19.
    University of California San Diego School of Medicine, La Jolla, CA, USA.
    Background. Systemic lupus erythematosus (SLE) is a chronic, inflammatory, connective tissue disease that commonly affects the joints and a variety of organs due to an overactivation of the body's immune system. There is wide heterogeneity in presentation of SLE patients, including lung, central nervous system, skin, kidney, and hematologic manifestations. Read More

    Systemic alterations and their oral manifestations in pregnant women.
    J Obstet Gynaecol Res 2017 Jan;43(1):16-22
    Clinica Odontologica, Federal University of Maranhao, São Luís, Brazil.
    The aims of this literature review are: to depict the main oral diseases that are related to pregnancy; to clarify some of the possible systemic mechanisms that are associated with these changes; and to address issues about oral care during pregnancy. A woman's organs undergo various physiological, neurological, and hormonal changes during pregnancy. Such changes occur gradually and are essential for the development of the fetus, providing what is needed for tissue formation and establishment of reserves for uterine and fetal life. Read More

    Oral manifestations of gastrointestinal diseases in children. Part 4: Coeliac disease.
    Eur J Paediatr Dent 2016 Dec;17(4):332-334
    Department of Surgical sciences and Integrated Diagnostics, University of Genova, Genoa, Italy.
    Alterations within the oral cavity can be the first sign of systemic diseases and may thus allow for an early diagnosis and treatment. In particular, being the oral cavity part of the gastrointestinal system, oral alterations can be an expression of a gastrointestinal disease. Dental enamel hypoplasia and aphthous ulcers have been found to be more common in children with coeliac disease compared with the general population and to regress after the patient is started on a gluten free-diet. Read More

    Orofacial granulomatosis as early manifestation of Crohn's disease: report of a case in a paediatric patient.
    Eur J Paediatr Dent 2016 Dec;17(4):318-321
    1st Department of Pediatrics, Medical School, University of Athens, Aghia Sophia Children's Hospital, Athens, Greece.
    Background: Orofacial granulomatosis (OFG) is a controversial entity mainly characterised by recurrent or permanent soft tissue swelling of sudden onset in the orofacial area with a histologic appearance of granulomatous inflammation. Differential diagnosis includes local diseases and systemic conditions, such as Crohn's disease (CD). A case of OFG in a paediatric patient is reported here, focusing on the clinical features, diagnostic procedures, treatment and long-term outcome. Read More

    Steinert syndrome and repercussions in dental medicine.
    Arch Oral Biol 2017 Mar 23;75:37-47. Epub 2016 Dec 23.
    Health Sciences Faculty, Fernando Pessoa University, Porto, Portugal. Electronic address:
    Steinert syndrome, also called myotonic dystrophy type 1, is a genetic disorder with autosomal dominant transmission characterized by myotonia and a multisystemic clinical picture that affects several tissues of the human body. The most common systemic phenotypes are: muscular, cardiac, respiratory, CNS, ocular, gynecological, digestive, orthopedical, as well as cognitive and psychological symptoms (cognitive decline). Muscles involved in voluntary movement are highly affected by myotonia especially distal muscles of upper limbs. Read More

    Dental and Anaesthetic Challenges in a Patient with Dystrophic Epidermolysis Bullosa.
    Sultan Qaboos Univ Med J 2016 Nov 30;16(4):e495-e499. Epub 2016 Nov 30.
    Oral & Maxillofacial Surgery Residency Program, Oman Medical Specialty Board, Muscat, Oman.
    Epidermolysis bullosa is a group of rare genetic disorders characterised by skin and mucous membrane fragility and systemic manifestations of variable severity. We report a case of dystrophic epidermolysis bullosa in an 18-year-old male patient who presented to the Department of Oral Health at Sultan Qaboos University Hospital, Muscat, Oman, in 2015 with recurrent dental pain and infections. Due to the poor dental status of the patient and anticipated operative difficulties due to microstomia and limited mouth opening, the patient underwent full dental clearance under general anaesthesia. Read More

    Mucocutaneous manifestations of Cowden's syndrome.
    Indian Dermatol Online J 2016 Nov-Dec;7(6):512-515
    Department of Oral Pathology and Microbiology, Mamata Dental College and Hospital, Khammam, Telangana, India.
    Cowden's syndrome is an autosomal dominant genodermatosis with variable orofacial and systemic manifestations. Here we present one such classical case of Cowden's syndrome in a 45-year-old female patient with features such as multiple cutaneous papillomatosis, oral fibromas, and fibromas involving multiple organs such as gastrointestinal tract (multiple polyps), thyroid disorders, and breast cancer. Read More

    Kawasaki disease for dermatologists.
    Indian Dermatol Online J 2016 Nov-Dec;7(6):461-470
    Allergy Immunology Unit, Department of Pediatrics, PGIMER, Chandigarh, India.
    Kawasaki disease (KD) is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. Read More

    Unusual maxillary osteoblastic and osteolytic lesions presenting as an initial manifestation of childhood acute myeloid leukemia: A case report.
    Quintessence Int 2017 ;48(2):149-153
    Changes in facial bones may represent a manifestation of systemic disease. Dentists play an important role in the early detection of these manifestations of complex systemic diseases. A case of unusual maxillary mixed (osteoblastic and osteolytic) lesions as an initial manifestation of childhood acute myeloid leukemia (AML) is presented. Read More

    Orofacial manifestations in patients with inflammatory rheumatic diseases.
    Best Pract Res Clin Rheumatol 2016 Oct 18;30(5):826-850. Epub 2016 Nov 18.
    Birmingham Behçet's Syndrome Centre of Excellence, Sheldon Block, Birmingham and Midland Eye Centre, City Hospital, Dudley Road, Birmingham, B18 7QH, UK. Electronic address:
    The main orofacial manifestation of the inflammatory rheumatic diseases is that of Sjögren's syndrome. In addition, there is a constellation of orofacial manifestations of the inflammatory rheumatic diseases, many of which are extra-articular with some constituting presenting signs of the underlying rheumatic disease. This review will discuss the orofacial manifestations in a variety of connective tissue diseases and will also allude to the oral adverse drug reactions that may occur as a consequence of therapy. Read More

    Sjögren Syndrome and Other Causes of Sicca in Older Adults.
    Clin Geriatr Med 2017 Feb;33(1):87-103
    National Institute of Dental and Craniofacial Research, National Institutes of Health, 10 Center Drive, Bethesda, MD 20892, USA.
    Dry eye and dry mouth symptoms are each reported by up to 30% of persons more than 65 years of age, particularly in women. Medication side effects are the most common contributing factors. The evaluation of these symptoms requires measures of ocular and oral dryness. Read More

    [Systemic lupus erythematosus : Unusual cutaneous manifestations].
    Hautarzt 2016 Dec;67(12):970-981
    Abteilung Dermatologie und Venerologie, Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Wien, Österreich.
    Background: Various different mucocutaneous symptoms may affect up to 80 % of systemic lupus erythematosus (SLE) patients.

    Objectives: To investigate, various unspecific, but otherwise typical clinical symptoms of skin and mucous membranes that arise in SLE patients other than those defined as SLE criteria such as butterfly rash, chronic cutaneous lupus erythematosus, oral ulcers, and increased photosensitivity.

    Materials And Methods: Extensive search of peer-reviewed scientific articles was performed, medical histories of several SLE patients seen in our department were analyzed, and the rare disease courses in three SLE patients are presented. Read More

    Adult-onset Still's disease-like manifestation accompanied by the cancer recurrence after long-term resting state.
    Mod Rheumatol 2016 Dec 9:1-5. Epub 2016 Dec 9.
    a The First Department of Internal Medicine, Division of Rheumatology and Nephrology , Kyorin University School of Medicine , Tokyo , Japan.
    A 72-year-old woman presented 9 months ago with skin rash on her bilateral forearms, which was followed by intermittent high fever, and stiffness and swelling of her bilateral fingers. She was diagnosed with seronegative rheumatoid arthritis (RA). She had a past history of breast cancer and had undergone breast preservation surgery 13 years previously. Read More

    Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.
    Lupus 2016 Nov 10. Epub 2016 Nov 10.
    Department of Pediatrics, Duke University Medical Center, Durham, USA.
    Objective: Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Read More

    First Real-World Insights into Belimumab Use and Outcomes in Routine Clinical Care of Systemic Lupus Erythematosus in Germany: Results from the OBSErve Germany Study.
    Rheumatol Ther 2016 Dec 1;3(2):271-290. Epub 2016 Nov 1.
    Medical Department, Immunology and Rare Diseases Business Unit, GlaxoSmithKline, Hamburg, Germany.
    OBSErve Germany was the first observational study of belimumab as add-on treatment for systemic lupus erythematosus (SLE) in routine clinical care in Germany, retrospectively collecting data from 102 SLE patients, 6 months before and after belimumab initiation. Most patients had moderate or severe SLE and several SLE manifestations. After 6 months of belimumab treatment, 78% of patients showed an improvement in overall disease activity of at least 20% in their physician's judgment and for 42% of patients the improvement was at least 50%. Read More

    Cranial Nerve-VI Palsy as the Main Clinical Manifestation of Neurosarcoidosis.
    Neurologist 2016 Nov;21(6):109-111
    *Associated Specialists Inc., United Hospital Center, Bridgeport, WV †Center for Advanced Health Care, Lehigh Valley Health Network, Allentown, PA ‡Neurological Institute of New Jersey, Rutgers, the State University of New Jersey, Newark, NJ.
    Introduction: Sarcoidosis is a chronic, systemic, inflammatory disorder that is characterized by the formation of noncaseating granulomas. Patients may present with cranial nerve palsy, paresthesia, paresis, pyramidal signs, progressive cognitive decline, urinary retention, seizures, or hypothalamic-pituitary syndrome. Although the diagnosis of neurosarcoidosis can be challenging, neurological manifestations of sarcoidosis occur more frequently than previously described. Read More

    Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
    Eur Respir J 2016 Nov 1;48(5):1429-1441. Epub 2016 Sep 1.
    Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre de référence des maladies orphelines pulmonaires; Université de Lyon, Université Lyon I; UCBL-INRA-ENVL-EPHE, UMR754; IFR128; Lyon, France.
    The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49. Read More

    Review of orofacial considerations of systemic sclerosis or scleroderma with report of analysis of 3 cases.
    Indian J Dent 2016 Sep;7(3):134-139
    Department of Oral Medicine and Radiology, SP Dental College, Wardha, Maharashtra, India.
    Scleroderma (skleros; hard, and derma; skin), is currently known as systemic sclerosis due to its progressive nature and widespread tissue involvement. It is a rare connective tissue disorder with a wide range of oral manifestations. Thickening of the skin is the hallmark of the disease. Read More

    Juvenile systemic lupus erythematosus in Nigeria.
    Lupus 2017 Mar 23;26(3):329-333. Epub 2016 Oct 23.
    3 Department of Medicine, University of Ilorin Teaching Hospital, Ilorin, Nigeria.
    Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Read More

    Orofacial manifestations of scleroderma. A literature review.
    Rev Stomatol Chir Maxillofac Chir Orale 2016 Nov 27;117(5):322-326. Epub 2016 Jul 27.
    Service de chirurgie maxillo-faciale, Hôpital de la Timone, 264, rue Saint-Pierre, 13385 Marseille, France; Aix-Marseille Université, Jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France; Aix Marseille Université, CNRS, LPL UMR 7309, 13100 Aix-en-Provence, France.
    Introduction: Scleroderma is a rare disease of the connective tissue (50 to 200 patients/1 million people; 60,000 patients in France). We conducted a literature review about the orofacial manifestations of scleroderma that have been little studied.

    Material And Methods: The 45 articles found in 6 different databases by using the keywords "scleroderma", "systemic sclerosis", "oral medicine", "face" and published between 1944 and 2016 were selected, for a total of 328 patients. Read More

    Concomitant herpes simplex virus colitis and hepatitis in a man with ulcerative colitis: Case report and review of the literature.
    Medicine (Baltimore) 2016 Oct;95(42):e5082
    aDivision of Infectious Diseases, Emory University School of Medicine bDepartment of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital cDivision of Digestive Diseases, Emory University School of Medicine dEmory Transplant Center, Atlanta, GA.
    Background: Herpesvirus infections often complicate the clinical course of patients with inflammatory bowel disease; however, invasive disease due to herpes simplex virus is distinctly uncommon.

    Methods: We present a case of herpes simplex virus colitis and hepatitis, review all the previously published cases of herpes simplex virus colitis, and discuss common clinical features and outcomes. We also discuss the epidemiology, clinical manifestations, diagnosis, and management of herpes simplex virus infections, focusing specifically on patients with inflammatory bowel disease. Read More

    Oral manifestations of gastrointestinal diseases in children. Part 3: Ulcerative colitis and gastro-oesophageal reflux disease.
    Eur J Paediatr Dent 2016 Sep;17(3):248-250
    Department of Paediatrics, University of Milan, Ospedale dei Bambini Vittore Buzzi, Milan, Italy.
    Background: Alterations of the oral cavity are common in children: 22% of children aged less than 4 years and 44% of those aged more than 12 develop dental erosion, 9-95% of children in Europe and in North America develop gingivitis, with adolescents showing a prevalence of more than 60% [Italian Ministry of Health, Guidelines 2013]. Alterations within the oral cavity can be the first sign of systemic diseases and may thus allow for an early diagnosis and treatment. In particular, being the oral cavity a part of the gastrointestinal system, oral alterations can be an expression of a gastrointestinal disease. Read More

    The impact of hepatitis C virus outside the liver: Evidence from Asia.
    Liver Int 2017 Feb 10;37(2):159-172. Epub 2016 Nov 10.
    Department of Internal Medicine and Therapeutics, University of Pavia, Pavia, Italy.
    Between 80 and 115 million people worldwide are chronically infected with hepatitis C virus, with 60%-90% of these being undiagnosed. Untreated chronic hepatitis C (CHC) is associated with progressive liver disease, cirrhosis, hepatocellular carcinoma and liver-related mortality. A number of extrahepatic manifestations are also reported in CHC patients, further adding to the burden of the disease. Read More

    Multiple Roles for B-Lymphocytes in Sjogren's Syndrome.
    J Clin Med 2016 Oct 8;5(10). Epub 2016 Oct 8.
    College of Veterinary Medicine University of Florida, Gainesville, FL 32608, USA.
    Sjogren's syndrome (SS) is a complex heterogeneous autoimmune disease resulting in loss of salivary gland and lacrimal gland function that may include multiple systemic manifestations including lymphoma. Multiple cell types participate in disease pathogenesis. This review discusses evidence for abnormal B cell subpopulations in patients with SS, critical roles of B cells in SS and the status of B cell-directed therapies in the management of patients with SS. Read More

    Management of patients with ocular manifestations in vesiculobullous disorders affecting the mouth.
    Oral Dis 2016 Oct 7. Epub 2016 Oct 7.
    Department of Ophthalmology, Rigshospitalet, University Hospital of Copenhagen, Copenhagen, Denmark.
    Pemphigoid and pemphigus diseases as well as Stevens-Johnson syndrome present as vesiculobullous disorders of the skin and may additionally involve both the oral cavity and the ocular surface. Ocular involvement ranges from mild irritation and dry eye disease to chronic conjunctivitis, symblepharon, eyelid malposition, ocular surface scarring and severe visual loss. In addition to diagnostic assessments, ophthalmologists must treat the dry eye and meibomian gland dysfunction components of these diseases using a stepladder approach, including eyelid hygiene and lubricants. Read More

    What rheumatologists should know about orofacial manifestations of autoimmune rheumatic diseases.
    Rev Bras Reumatol Engl Ed 2016 Sep - Oct;56(5):441-450. Epub 2016 Mar 16.
    Programa de Pós-Graduação em Ciências Médicas, Faculdade de Medicina, Universidade de Brasília (UnB), Brasília, DF, Brazil.
    Orofacial manifestations occur frequently in rheumatic diseases and usually represent early signs of disease or of its activity that are still neglected in clinical practice. Among the autoimmune rheumatic diseases with potential for oral manifestations, rheumatoid arthritis (RA), inflammatory myopathies (IM), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), relapsing polychondritis (RP) and Sjögren's syndrome (SS) can be cited. Signs and symptoms such as oral hyposalivation, xerostomia, temporomandibular joint disorders, lesions of the oral mucosa, periodontal disease, dysphagia, and dysphonia may be the first expression of these rheumatic diseases. Read More

    Orofacial manifestations of systemic sclerosis.
    Br Dent J 2016 Sep;221(6):305-10
    Royal National Hospital for Rheumatic Diseases, Bath and Visiting Senior Lecturer, Department of Pharmacy and Pharmacology, University of Bath.
    Systemic sclerosis (SSc) is a multisystem disease of unknown aetiology characterised by microangiopathy, dysregulated immune function and tissue remodelling, which commonly involves the oral cavity. Orofacial manifestations of SSc contribute greatly to overall disease burden and yet are regularly overlooked and under-treated. This may reflect a pre-occupation amongst rheumatology clinicians on potentially life-threatening internal organ involvement, but is also a consequence of insufficient engagement between rheumatologists and dental professionals. Read More

    Solitary Plasmacytoma of the Mandible: An Uncommon Entity.
    Iran Red Crescent Med J 2016 Jul 20;18(7):e22932. Epub 2016 Feb 20.
    Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Baskent University, Bahcelievler, Ankara, Turkey.
    Introduction: Plasma cell dyscrasias are characterized by a monoclonal neoplastic proliferation of plasma cells. Solitary bone plasmacytoma (SBP) is a local form of the disease with the vertebrae and long bones being the most frequently encountered sites. Its prevalence in the maxillofacial area is extremely rare. Read More

    Derivation and Validation of the Periodontal and Tooth Profile Classification System for Patient Stratification.
    J Periodontol 2017 Feb 13;88(2):153-165. Epub 2016 Sep 13.
    Department of Periodontology, School of Dentistry, University of North Carolina at Chapel Hill, Chapel Hill, NC.
    Background: The goal of this study is to use bioinformatics tools to explore identification and definition of distinct periodontal and tooth profile classes (PPCs/TPCs) among a cohort of individuals by using detailed clinical measures at the tooth level, including both periodontal measurements and tooth loss.

    Methods: Full-mouth clinical periodontal measurements (seven clinical parameters) from 6,793 individuals from the Dental Atherosclerosis Risk in Communities Study (DARIC) were used to identify PPC. A custom latent class analysis (LCA) procedure was developed to identify clinically distinct PPCs and TPCs. Read More

    A 3 Year-Old Male Child Ingested Approximately 750 Grams of Elemental Mercury.
    Balkan Med J 2016 Jul 1;33(4):467-9. Epub 2016 Jul 1.
    Department of Pediatrics, İstanbul University İstanbul School of Medicine, İstanbul, Turkey.
    Background: The oral ingestion of elemental mercury is unlikely to cause systemic toxicity, as it is poorly absorbed through the gastrointestinal system. However, abnormal gastrointestinal function or anatomy may allow elemental mercury into the bloodstream and the peritoneal space. Systemic effects of massive oral intake of mercury have rarely been reported. Read More

    Heat shock protein-based therapy as a potential candidate for treating the sphingolipidoses.
    Sci Transl Med 2016 Sep;8(355):355ra118
    Department of Pharmacology, University of Oxford, Oxford, U.K.
    Lysosomal storage diseases (LSDs) often manifest with severe systemic and central nervous system (CNS) symptoms. The existing treatment options are limited and have no or only modest efficacy against neurological manifestations of disease. We demonstrate that recombinant human heat shock protein 70 (HSP70) improves the binding of several sphingolipid-degrading enzymes to their essential cofactor bis(monoacyl)glycerophosphate in vitro. Read More

    Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab: a case report.
    Ital J Pediatr 2016 Sep 6;42(1):81. Epub 2016 Sep 6.
    Department of Pediatrics, University of Chieti, Via dei Vestini 5, Chieti, 66100, Italy.
    Background: Autoimmune sensorineural hearing loss, also known as autoimmune inner ear disease (AIED) is a rare clinical entity characterized by progressive and bilateral sensorineural hearing loss often accompanied by vestibular symptoms. Diagnosis is essential as a consistent number of patients show a positive response to steroids alone or in association with other immunosuppressive drugs. AIED is defined as primary when the disease is limited to the ear, whereas in up to a third of cases it is associated to other systemic autoimmune diseases such as Behçet disease (BD). Read More

    [Inflammatory diseases of oral mucous membranes].
    Hautarzt 2016 Oct;67(10):786-792
    Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität, Frauenlobstr. 9-11, 80337, München, Deutschland.
    Background: Mucous membranes are involved in many different inflammatory disorders.

    Methods: We describe the clinical features, diagnostic workup, and therapy of inflammatory diseases of mucous membranes.

    Results: The diagnostic workup is based on medical history, clinical manifestations, histopathology, and serology. Read More

    Gene therapy improves dental manifestations in hypophosphatasia model mice.
    J Periodontal Res 2016 Aug 26. Epub 2016 Aug 26.
    Osaka University Graduate School of Dentistry, Osaka, Japan.
    Background And Objective: Hypophosphatasia is a rare inherited skeletal disorder characterized by defective bone mineralization and deficiency of tissue non-specific alkaline phosphatase (TNSALP) activity. The disease is caused by mutations in the liver/bone/kidney alkaline phosphatase gene (ALPL) encoding TNSALP. Early exfoliation of primary teeth owing to disturbed cementum formation, periodontal ligament weakness and alveolar bone resorption are major complications encountered in oral findings, and discovery of early loss of primary teeth in a dental examination often leads to early diagnosis of hypophosphatasia. Read More

    Genetics and pathophysiology of granulomatosis with polyangiitis (GPA) and its main autoantigen proteinase 3.
    Mol Cell Probes 2016 Dec 21;30(6):366-373. Epub 2016 Aug 21.
    Department of Internal Medicine, Division of Rheumatology and Clinical Immunology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany; ACURA Rheumatology Center, Bad Kreuznach, Germany.
    Granulomatosis with polyangiitis (GPA) is a severe autoimmune disease and one of the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although its etiology and pathophysiology are still widely unknown, it is accepted that infections, environmental factors, epigenetic modifications, and a genetic predisposition provide the basis for this systemic disorder. GPA typically evolves into two phases: an initial phase characterized by ear, nose and throat (ENT) manifestations, such as chronic sinusitis and otitis, ulceration of the oral cavity and pharynx, as well as pulmonary nodules and a severe generalized phase, defined by the occurrence of rapidly progressive glomerulonephritis, pulmonary hemorrhage, and arthritis. Read More

    Ocular Onset of Paraneoplastic Pemphigus Presenting as Hyperemic Conjunctivitis and Massive Bilateral Eyelid Ulceration: A Case Report and Literature Review.
    Ocul Immunol Inflamm 2016 Aug 18:1-4. Epub 2016 Aug 18.
    a Department of Ophthalmology , Humanitas Clinical and Research Institute , Rozzano , Milan , Italy.
    Introduction: Paraneoplastic clinical signs are characterized by a large and heterogeneous variety of manifestations due to several possible underlying neoplasms. Paraneoplastic pemphigus (PNP) is a particular paraneoplastic variety that usually primarily affects the dermic and/or oral mucosa and is characterized by a high rate of mortality (90%). Therefore, it is important to recognize its possible signs early. Read More

    Asthma and Rhinitis Induced by Selective Immediate Reactions to Paracetamol and Non-steroidal Anti-inflammatory Drugs in Aspirin Tolerant Subjects.
    Front Pharmacol 2016 20;7:215. Epub 2016 Jul 20.
    Allergy Service, Infanta Leonor University Hospital Madrid, Spain.
    In subjects with non-steroidal anti-inflammatory drugs (NSAIDs)- exacerbated respiratory disease (NERD) symptoms are triggered by acetyl salicylic acid (ASA) and other strong COX-1 inhibitors, and in some cases by weak COX-1 or by selective COX-2 inhibitors. The mechanism involved is related to prostaglandin pathway inhibition and leukotriene release. Subjects who react to a single NSAID and tolerate others are considered selective responders, and often present urticaria and/or angioedema and anaphylaxis (SNIUAA). Read More

    Gingival hyperplasia as first sign of recurrence of granulomatosis with polyangiitis (Wegener's granulomatosis): case report and review of the literature.
    BMC Oral Health 2016 Aug 2;17(1):33. Epub 2016 Aug 2.
    Department of Cranio-Maxillofacial Surgery, University Hospital Münster, Albert-Schweitzer-Campus 1, Gebäude W 30, D-48149, Münster, Germany.
    Background: Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener's granulomatosis, is a rare systemic disease of unknown etiology which can affect all areas of the body, including the oral cavity. The typical oral manifestations occur as nonspecific erosive/ulcerative lesions of the oral cavity or appear with hyperplastic gingivitis, a so called "strawberry gingivitis".

    Case Presentation: We report here about an extremely rare case with hyperplastic gingivitis as the first sign of recurrence of GPA in the absence of oral manifestations in the primary disease. Read More

    Impact of sex disparities on the clinical manifestations in patients with systemic lupus erythematosus: A systematic review and meta-analysis.
    Medicine (Baltimore) 2016 Jul;95(29):e4272
    Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, Hunan, People's Republic of China.
    Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune multiorgan disorder of unknown etiology. It affects both men and women, but with different disease manifestations of differing disease severity and in varying proportion, with a female predominance of approximately 90%. There have been numerous studies addressing this issue, especially its implications in relation to optimal sex-tailored treatment and improvement of survival rate; however, further research is warranted. Read More

    Ocular Manifestations of Inherited Phospholipase-Cγ2-Associated Antibody Deficiency and Immune Dysregulation.
    Cornea 2016 Jul 20. Epub 2016 Jul 20.
    *Schepens Eye Research Institute, Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, Harvard Medical School, Boston, MA; and †Columbia University, Edward S. Harkness Eye Institute, New York, NY.
    Purpose: To report the ocular manifestations of phospholipase-Cγ2-associated antibody deficiency and immune dysregulation (PLAID).

    Methods: Case report and literature review.

    Results: A 21-year-old woman diagnosed with PLAID was referred for evaluation of repeated episodes of ocular inflammation resulting in bilateral peripheral corneal pannus with episcleritis and corneal scarring accompanied by systemic manifestations including epidermolysis bullosa and interstitial lung disease. Read More

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