Clin Rev Allergy Immunol 2017 Jun 23. Epub 2017 Jun 23.

Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland.

Inflammatory bowel disease (IBD) with its two main subtypes Crohn's disease and ulcerative colitis is not restricted to the gastrointestinal tract. Indeed, so-called extraintestinal manifestations (EIMs) are frequent and considerably affect morbidity and mortality. The prevalence of EIMs ranges from 6 to 47%. Read More

BMC Infect Dis 2017 Jun 20;17(1):441. Epub 2017 Jun 20.

Division of Infectious Diseases, Department of Internal Medicine, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, 5 Fuhsing Street, Kueishan, Taoyuan, 333, Taiwan.

Background: Acute hepatitis A is a fecal-oral transmitted disease related to inadequate sanitary conditions. In addition to its traditional classification, several outbreaks in the men who have sex with men (MSM) population have resulted in acute hepatitis A being recognized as a sexually transmitted disease. However, few studies have clarified the clinical manifestations in these outbreaks involving the MSM population. Read More

Ter Arkh 2017 ;89(5):26-32

Moscow Research Institute of Psychiatry, Branch, V.P. Serbsky Federal Medical Research Center of Psychiatry and Narcology, Ministry of Health of Russia, Moscow, Russia.

Aim: To analyze of the prevalence of stressful factors and mental disorders (MDs), as well as their clinical psychopathological and clinical psychological characteristics to improve the comprehensive diagnosis and treatment of systemic scleroderma (SSD).

Subjects And Methods: Examinations were performed in 110 patients (predominantly women (n=97 (88.2%); mean age, 49. Read More

Nutrients 2017 Jun 16;9(6). Epub 2017 Jun 16.

Unit of Immunodiagnostic and Molecular Pathology, Department of Medical, Oral and Biotechnological Sciences, University "G. d'Annunzio" Chieti-Pescara, 66100 Chieti, Italy.

Microbiota play a key role in various body functions, as well as in physiological, metabolic, and immunological processes, through different mechanisms such as the regulation of the development and/or functions of different types of immune cells in the intestines. Evidence indicates that alteration in the gut microbiota can influence infectious and non-infectious diseases. Bacteria that reside on the mucosal surface or within the mucus layer interact with the host immune system, thus, a healthy gut microbiota is essential for the development of mucosal immunity. Read More

Arthritis Res Ther 2017 Jun 15;19(1):138. Epub 2017 Jun 15.

Rheumatology Research Group, Vall d'Hebron Research Institute, Barcelona, 08035, Spain.

Background: Systemic lupus erythematosus (SLE) is a genetically complex rheumatic disease characterized by heterogeneous clinical manifestations of unknown etiology. Recent studies have suggested the existence of a genetic basis for SLE heterogeneity. The objective of the present study was to identify new genetic variation associated with the clinically relevant phenotypes in SLE. Read More

Dtsch Arztebl Int 2017 May;114(20):354-361

Charité Center for Internal Medicine, Medical Department-Division of Rheumatology and Clinical Immunology, Berlin, Germany; University Clinic of Rheumatology, Immunology and Allergology, Inselspital Bern, Bern, Switzerland; BfA Wendelstein Rehabilitation Clinic, Rheumatology Center, AHB Clinic, Bad Aibling, Germany; Department of Ophthalmology, Charité Campus Virchow, Berlin, Germany; Oral Surgery, The School of Dentistry, University of Birmingham, United Kingdom.

Background: Sjögren's syndrome is one of the more common inflammatory rheumatological diseases, with a prevalence of at least 0.4% in Germany.

Methods: This review is based on pertinent articles retrieved by a selective search in PubMed. Read More

Exp Ther Med 2017 Jun 28;13(6):3337-3340. Epub 2017 Apr 28.

Department of Ophthalmology, The First Hospital of Jilin University, Changchun, Jilin 130021, P.R. China.

Granulomatosis with polyangiitis (GPA) is a systemic form of vasculitis that involves small to medium sized vessels and is associated with high morbidity and mortality. GPA presents a continuous and difficult clinical diagnostic concern, due to the rarity of the disease and the diversity of the manifestations. This case report discusses the unusual symptoms presented by a particular patient, discusses these manifestations and explains how the final diagnosis was identified as GPA. Read More

J Clin Diagn Res 2017 Apr 1;11(4):ZD19-ZD20. Epub 2017 Apr 1.

Senior Lecturer, Department of Oral Medicine and Radiology, Maharana Pratap College of Dentistry and Research Centre, Gwalior, Madhya Pradesh, India.

Leprosy is a contagious and granulomatous disease which is caused by Mycobacterium leprae. The incubation period for leprosy is five to seven years and it can take as long as about 30 years before signs and symptoms of leprosy develop in some patients. It is a chronic systemic disease which mostly affects the skin and peripheral nerves. Read More

Reumatologia 2017 28;55(2):79-83. Epub 2017 Apr 28.

Medical Biology Research Center, Kermanshah University of Medical Sciences, Iran.

Objectives: The pathergy skin test is a hypersensitivity reaction to a prick skin trauma caused by a pin or a needle, which is considered as a specific presentation in Behçet's disease (BD) and the precise mechanism of this test is not well elucidated. This study was designed to evaluate the association of pathergy reaction (PR) with the active clinical manifestations of BD patients, to assess the clinical importance of PR.

Materials And Methods: This was a cohort study on 1675 BD patients who fulfilled the entry criteria based on the International Criteria for Behçet's Disease (ICBD) from 1975 to 2011. Read More

Oral Health Prev Dent 2017 May 19. Epub 2017 May 19.

Purpose: Crohn's disease (CD) is a chronic inflammatory bowel disease characterised by localised areas of nonspecific, noncaseating granulomas. Approximately 1/3 of CD patients develop extraintestinal manifestations in the course of their disease. This review focuses on oral manifestations of CD to understand if oral lesions could help clinicians in the diagnosis of systemic CD. Read More

Am J Emerg Med 2017 Apr 29. Epub 2017 Apr 29.

Cardiology, Lincoln Medical and Mental Health Center, United States.

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease which follows a relapsing and remitting course that can manifest in any organ system. While classic manifestations consist of arthralgia, myalgia, frank arthritis, a malar rash and renal failure to name a few, cardiac tamponade, however, is a far less common and far more dangerous presentation. We highlight the case of a 61year-old male with complaints of acute onset shortness of breath and generalized body aches associated with a fever and chills in the ER. Read More

Clin Immunol 2017 May 17. Epub 2017 May 17.

Department of Oral Biology, School of Dental Medicine, University of Buffalo, The State University of New York, Buffalo, NY 14214, USA; Autoimmune Division, Trinity Biotech, 60 Pineview Drive, Buffalo, NY 14228, USA; Department of Oral Diagnostics Sciences, School of Dental Medicine, University of Buffalo, The State University of New York, Buffalo, NY 14214, USA. Electronic address:

Animal models that recapitulate human disease are crucial for the study of Sjögren's Syndrome (SS). While several SS mouse models exist, there are few primary SS (pSS) models that mimic systemic disease manifestations seen in humans. Similar to pSS patients, NOD. Read More

Intest Res 2017 Apr 27;15(2):255-259. Epub 2017 Apr 27.

Department of Pathology, Kyungpook National University School of Medicine, Daegu, Korea.

A 31-year-old woman with a 15-year history of Takayasu's arteritis (TA) and a 13-year history of Hashimoto's thyroiditis presented with hematochezia. She received a diagnosis of Sjögren's syndrome at 1 month before her visit to Kyungpook National University Medical Center. Her colonoscopic findings were compatible with a diagnosis of ulcerative colitis (UC). Read More

Nat Rev Dis Primers 2017 May 11;3:17026. Epub 2017 May 11.

Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.

Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. Read More

Am J Clin Dermatol 2017 May 5. Epub 2017 May 5.

Center of Excellence in Immunology and Immune-mediated Disease, Faculty of Medicine, Chulalongkorn University, Rama 4 Road, Pathumwan, Bangkok, 10330, Thailand.

Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Read More

Allergy Asthma Clin Immunol 2017 24;13:21. Epub 2017 Apr 24.

Consulta Multidisciplinar de Linfomas Cutâneos e Mastocitoses (CMLC), Hospital de Santo António (HSA), Centro Hospitalar do Porto (CHP), Porto, Portugal.

Background: Mastocytosis are rare diseases characterized by an accumulation of clonal mast cells (MCs) in one or multiple organs or tissues. Patients with systemic mastocytosis (SM), whose MCs frequently arbor the activating D816V KIT mutation, may have indolent to aggressive diseases, and they may experience MC mediator related symptoms. Indolent SM with recurrent anaphylaxis or vascular collapse in the absence of skin lesions, ISMs(-), is a specific subtype indolent SM (ISM), and this clonal MC activation disorder represents a significant fraction of all MC activation syndromes. Read More

Clin Immunol 2017 Apr 8. Epub 2017 Apr 8.

Department of Oral Biology, School of Dental Medicine, State University of New York at Buffalo, Buffalo, NY 14214, United States; Department of Oral Diagnostic Sciences, School of Dental Medicine, State University of New York at Buffalo, Buffalo, NY 14214, United States. Electronic address:

Sjögren's syndrome (SS) is an autoimmune disease of exocrine tissue that primarily affects women. Although patients typically experience xerostomia and xerophthalmia, numerous systemic disease manifestations are seen. Innate immune hyperactivity is integral to many autoimmune diseases, including SS. Read More

Int J Med Sci 2017 23;14(3):191-200. Epub 2017 Feb 23.

Department of Internal Medicine, Division of Clinical Immunology;; Department of Immunology, Erasmus MC, Rotterdam, The Netherlands.

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx and/or vagina. Extraglandular manifestations may also be prevalent in patients with pSS, including cutaneous, musculoskeletal, pulmonary, renal, hematological and neurological involvement. Read More

Front Immunol 2017 7;8:241. Epub 2017 Mar 7.

Faculty of Medicine and Dentistry, Department of Dentistry, University of Alberta, Edmonton, AB, Canada; Faculty of Medicine and Dentistry, Department of Medical Microbiology and Immunology, University of Alberta, Edmonton, AB, Canada.

Mucosal surfaces account for the vast majority of HIV transmission. In adults, HIV transmission occurs mainly by vaginal and rectal routes but rarely via oral route. By contrast, pediatric HIV infections could be as the result of oral route by breastfeeding. Read More

Dent Clin North Am 2017 Apr;61(2):351-363

Department of Oral and Maxillofacial Surgery, Woodhull Medical Center, 760 Broadway, Room 2C-320, Brooklyn, NY 11206, USA.

This article focuses on common viral infections in the oral cavity with associated systemic manifestations. Discussed are the clinical features, histopathology, diagnosis, treatment, and prevention of viral infections in oral cavity. This will be a useful aid for general practitioners and other dental personnel wanting to expand their pathologic knowledge. Read More

Clin J Am Soc Nephrol 2017 Mar 16. Epub 2017 Mar 16.

Renal Disease and Hypertension, Pediatric Nephrology and Hypertension, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, Texas

Tuberous sclerosis complex is an autosomal dominant genetic disease characterized by growth of benign tumors (hamartomas) in multiple organs, especially the kidneys, brain, heart, lungs, and skin. Tuberous sclerosis complex is usually caused by a mutation in either the tuberous sclerosis complex 1 or tuberous sclerosis complex 2 gene, resulting in constitutive activation of mammalian target of rapamycin signaling. Currently, mammalian target of rapamycin inhibitors are recommended in adult patients with tuberous sclerosis complex for the treatment of asymptomatic, growing renal angiomyolipoma that are >3 cm in diameter and pediatric or adult patients with brain lesions (subependymal giant cell astrocytoma) that either are growing or are not amenable to surgical resection. Read More

Ann R Coll Surg Engl 2017 May 16;99(5):e135-e136. Epub 2017 Mar 16.

Mid Essex Hospital Services NHS Trust , UK.

An oral lesion as the first clinical presentation of sarcoidosis is extremely rare. We present the case of a 39-year-old woman who was referred to the oral and maxillofacial surgery department with a persistent asymptomatic nodular lesion in the hard palate. This was located adjacent to a grossly carious upper first molar and a provisional diagnosis of chronic periapical granuloma was made. Read More

Anesth Essays Res 2017 Jan-Mar;11(1):233-237

Department of Anaesthesiology and Critical Care, NIMS Medical College and Hospital, Jaipur, Rajasthan, India.

Aim: The aim of this article is to provide a view of amyloidosis and discuss implications for the anesthetic management of patients with this condition.

Material And Method: Urine samples from patients with plasma cell dyscrasias were obtained from a urine bank that gathers urine samples from patients who gave research use consent for specimens that would otherwise be considered waste.

Results: Patients with amyloidosis may present to the anesthesiologist for procedures relating to diagnosis, surgery relating to the underlying condition (e. Read More

J Rheumatol 2017 May 15;44(5):619-625. Epub 2017 Mar 15.

From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yıldız Technical University; Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Objective: Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE).

Methods: For the study, 719 patients (76. Read More

Mil Med 2017 Mar;182(3):e1843-e1848

Department of Preventive Medicine and Public Health, Blanchfield Army Community Hospital, 2576 23rd Street, Fort Campbell, KY 42223.

Introduction: Despite greater than 60,000 nonfatal firearm injuries per year in the United States, retained shrapnel is a relatively rare cause of systemic lead toxicity with less than 100 cases reported in the medical literature since 1867. While intra-articular retained shrapnel as a cause of lead toxicity is well-described, extra-articular fragments are less well known to cause symptomatic disease.

Case Report: A 31-year-old man initially presented with abdominal pain, constipation, jaundice, and elevated liver transaminases approximately 3 weeks after suffering a left lower extremity injury during athletic activity. Read More

Front Immunol 2017 27;8:200. Epub 2017 Feb 27.

Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari Aldo Moro , Bari , Italy.

Behçet's disease (BD) is a multi-systemic inflammatory disorder consisting of recurrent oral aphthosis, genital ulcers, and chronic relapsing bilateral uveitis; however, many other organs may be affected. Several pro-inflammatory cytokines, mainly derived from Th1 and Th17 lymphocytes, seem to be involved in different pathogenic pathways leading to development of the clinical manifestations. On this basis, the primary aim of our study was to compare a core set of pro-inflammatory cytokines between patients with BD and healthy control (HC). Read More

Head Neck Pathol 2017 Mar 8. Epub 2017 Mar 8.

Oral and Maxillofacial Pathologist, Department of Stomatology, Faculty of Dentistry, Universite de Montreal, Montreal, QC, Canada.

Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The condition is characterized by the appearance of one or more necrotic ulcers with a ragged undermined violaceous border and surrounding erythema. Lesions are often initiated by minor trauma. Read More

Clin Exp Rheumatol 2017 Feb 24. Epub 2017 Feb 24.

Division of Rheumatology, Department of Medicine, Joseph's Health Care, London, Canada.

Objectives: To determine the prevalence of and risk factors for avascular necrosis (AVN) in systemic lupus erythematosus (SLE).

Methods: MEDLINE, CINAHL, Web of Science, EMBASE and Cochrane Library were searched from inception to July, 2015 and a random effects model was used to combine frequencies; study quality was assessed using STROBE.

Results: 2,041 citations identified 62 articles. Read More

Curr Allergy Asthma Rep 2017 Feb;17(2):11

Department of Otorhinolaryngology, School of Medicine, University of Crete, Heraklion, Crete, Greece.

Purpose Of Review: Exogenously administered corticosteroids are widely used today in the field of rhinology. Allergic rhinitis (AR), non-allergic rhinitis (NAR), acute rhinosinusitis (ARS), chronic rhinosinusitis with (CRSwNP) and without (CRSsNP) nasal polyps, and autoimmune disorders with nasal manifestations are common diseases treated effectively with intranasal and oral glucocorticoids. We focus on physiological pathways, therapeutic benefits, indications, contra-indications, and side effects of glucocorticoid utilization in the treatment of rhinologic disorders such as AR, NAR, ARS, CRSsNP, and CRSwNP. Read More

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

Am J Ophthalmol 2017 Apr 30;176:141-147. Epub 2017 Jan 30.

The Rotterdam Eye Hospital, Rotterdam, Netherlands.

Purpose: To report intraocular manifestations of Erdheim-Chester Disease (ECD) with multimodal imaging.

Design: A retrospective observational case series.

Methods: This was a multicenter case series of 3 patients with confirmed tissue diagnosis of ECD that showed intraocular manifestations and were imaged at baseline and follow-up visits. Read More

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

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

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

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 Dis 2017 May 22;23(4):498-504. Epub 2017 Feb 22.

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

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

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

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

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

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

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

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

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

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

Clin Dermatol 2016 Nov - Dec;34(6):678-689. Epub 2016 Jul 9.

Assistant Professor of Dermatology, University of Connecticut School of Medicine, Farmington, CT.

Morphea, dermatomyositis (DM), and discoid lupus erythematosus (DLE) are autoimmune collagen vascular diseases that can present at any age. In all three of these diseases, the tenants of diagnosis and treatment are largely the same in both children and adults, with a few notable differences. Children with morphea are more likely to present with the linear subtype and have a higher incidence of extracutaneous manifestations. Read More

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

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

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

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