Sjögren's syndrome is currently considered an "autoimmune epithelitis," as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren's syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases. Further, xerophtalmia (also known as dry eye) frequently associated with keratoconjunctivitis sicca cumulatively affects approximately 10-30% of the general population with increasing incidence with age and is more frequently secondary to non-autoimmune diseases. Read More
A 46-year-old woman with a 30 pack-year smoking history presented with a worsening eruption on the left cheek that failed to improve with metronidazole gel. The cutaneous eruption spread to most of her face and did not respond to a brief tapering course of prednisone. During the initial evaluation at our institution, approximately 6 weeks after the onset of the cutaneous eruption, the patient had erythematous, crusted plaques on her face and scalp (Figure 1A); they were also present on the V-area of the anterior aspect of the neck and upper region of the chest, the shoulders, and the arms, with isolated lesions on the trunk and legs. Read More
Porphyrias are a group of metabolic disorders, which result from a specific abnormality in one of the eight enzymes of the heme biosynthetic pathway. These have been subdivided based on the predominant site of enzyme defect into hepatic and erythropoietic types and based on clinical presentation into acute neurovisceral and cutaneous blistering porphyrias. This review focuses on hepatic porphyrias, which include acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), aminolevulinic acid dehydratase deficiency porphyria (ADP), and porphyria cutanea tarda (PCT). Read More
Objectives: To determine the relationship between vascular biomarkers reflecting the vascular injury and organ involvement in systemic sclerosis (SSc).
Methods: Seventy-two SSc patients (66 female) fulfilling 2013 ACR/EULAR Criteria were evaluated. Serum samples of patients were collected for flow-cytometric analysis of sCD40L, tPA, MCP-1, sE-selectin, IL-8, IL-6, VEGF, sP-selectin, TGF-β1 and VCAM levels (Bender MedSystems) in SSc patients and 20 healthy controls. Read More
Mariabeatrice Principi, Antonella Contaldo, Andrea Iannone, Giuseppe Losurdo, Michele Barone, Enzo Ierardi, Alfredo Di Leo, Gastroenterology Section, Department of Emergency and Organ Transplantation, University of Bari, 70124 Bari, Italy.
Inflammatory bowel disease (IBD) could be associated with several extra-intestinal manifestations (EIMs) involving musculoskeletal, hepatopancreatobiliary, ocular, renal, and pulmonary systems, as well as the skin. In the last years, hidradenitis suppurativa (HS) is acquiring an increasing interest. IBD, especially Crohn's disease (CD), is among the most reported associated diseases in HS patients. Read More
Cutaneous lupus erythematosus (LE) is an autoimmune disease. The most common clinical forms are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE) and discoid LE (DLE). Cutaneous LE, mainly ACLE, can be the first sign of systemic LE (SLE). Read More
Introduction: Pseudoxanthoma elasticum (PXE), a multisystem orphan disease, clinically affects the skin, the eyes, and the cardiovascular system with considerable morbidity and mortality. The clinical manifestations reflect the underlying pathology consisting of ectopic mineralization of peripheral connective tissues.
Areas Covered: The diagnostic criteria of PXE include characteristic clinical findings, together with histopathology of accumulation of pleiomorphic elastic structures in the dermis with progressive mineralization, and the presence of mutations in the ABCC6 gene. Read More
Aims: To define the number/frequency of organ systems affected by extraintestinal manifestations (EIMs), to identify factors affecting the clinical course of inflammatory bowel disease (IBD) and EIM development, and to determine the impact of smoking, disease duration and location on the diagnosis of EIMs in Crohn's disease (CD) and ulcerative colitis (UC).
Methods: IBD patients were derived from a single university colorectal surgery practice. Smoking data were obtained through a modified Behavioral Risk Factor Surveillance System survey. Read More
Semin Arthritis Rheum 2014 Apr 9;43(5):681-8. Epub 2013 Oct 9.
Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France; Department of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.
Objectives: To describe the prevalence, characteristics, and outcome of cutaneous ischemia, and whether it can occur in systemic necrotizing vasculitides (SNVs), i.e., polyarteritis nodosa, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. Read More
Background And Aim: To identify predictive factors related to the development of erythema nodosum and pyoderma gangrenosum, in patients with inflammatory bowel disease (IBD).
Methods: Epidemiological and clinical data from 270 patients with Crohn's disease (CD) and 125 patients with ulcerative colitis (UC) were collected between 2003 and 2011. The variables retrospectively analyzed were: gender, age at diagnosis, type of IBD (CD or UC), smoking habit, pattern of disease (IBD), location and extension, family history, previous IBD-related surgery, other extraintestinal manifestations (EIMs), and previous biological and immunosuppressive therapy. Read More
Psoriasis is a multisystem disease. The cutaneous and musculoskeletal manifestations (psoriatic arthritis) are well recognized. However, the other manifestations of psoriatic disease including metabolic syndrome, atherosclerotic cardiovascular disease, depression, poor self-esteem, and self-destructive habits including obesity, smoking and excess alcohol consumption are underappreciated. Read More
Skin manifestations are frequently observed in lupus erythematosus (LE) and are manifold. Nonspecific and specific symptoms can be differentiated with the latter belonging to the dermatologically well-characterized clinical entities of acute cutaneous, subacute cutaneous, chronic cutaneous as well as intermittent cutaneous LE. These forms are differentially related to systemic LE. Read More
Objective: To examine the association between smoking and cutaneous involvement in systemic lupus erythematosus (SLE).
Methods: We analyzed data from a multicenter Canadian SLE cohort. Mucocutaneous involvement was recorded at the most recent visit using the Systemic Lupus Erythematosus Disease Activity Index 2000 Update (rash, alopecia, and oral ulcers), Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index (alopecia, extensive scarring, and skin ulceration), and the ACR revised criteria for SLE (malar rash, discoid rash, photosensitivity, and mucosal involvement). Read More
Introduction: Fabry disease is an X-linked inherited metabolic disorder characterized by the deficiency of lysosomal α-galactosidase A enzyme. This leads to the accumulation, into lysosomes through the body, of glycosphingolipids, mainly Gb3. Skin involvement and progressive multi-organ failure are usually observed. Read More
Scurvy is caused by prolonged dietary deficiency of vitamin C, the plasma concentration of which appears inversely related to mortality from all causes. Its clinical importance relates principally to its role as a cofactor in a number of enzyme reactions involved in collagen synthesis, dysfunction of which disrupts connective tissue integrity, resulting in impaired wound healing and capillary bleeding. In the UK, overt scurvy is diagnosed only rarely. Read More
Background: Hydroxychloroquine (HCQ) is not available in Japan. To design a clinical trial of HCQ, we evaluated the response to HCQ in Japanese patients with lupus-related skin disease using the cutaneous lupus erythematosus disease area and severity index (CLASI).
Methods: Twenty-seven patients with lupus-related skin disease who started HCQ at four hospitals were included. Read More
Clinical manifestations of lupus are encountered in a variety of disease entities, including isolated cutaneous lupus, undifferentiated connective tissue disease, mixed connective tissue disease, drug-induced lupus, overlap syndrome, and systemic lupus erythematosus (SLE). While each entity has been recognized as a specific disease with its own diverse clinical and serological pattern, one could argue that many findings are common. Could it be that all of these entities actually represent a spectrum of one disease? Could it be that rather than the genetic predisposition and hence controlled factors that govern this spectrum of diseases, that environmental factors associated with SLE could also play a role in the different entities of this spectrum? The traditional environmental triggers in SLE include sunlight and ultraviolet (UV) light, infections, smoking, and medications including biologics such as tumor necrosis factor alpha (TNF-a) blockers. Read More
In patients with cutaneous lupus erythematosus (CLE), it is important to provide instructions concerning methods of protection from sunlight and artificial sources of ultraviolet radiation. Topical corticosteroids are the mainstay of treatment for patients with CLE; however, they are of limited value because of their well-known side effects. Recently, calcineurin inhibitors have been shown to be efficient as topical therapy in various CLE subtypes. Read More
Gouty panniculitis is an unusual clinical manifestation of gout, characterized by the deposition of monosodium urate crystals in the lobular hypodermis. Its pathogenesis is poorly understood but is associated with hyperuricemia, and the clinical presence of indurate subcutaneous plaques, which may precede or appear subsequently to the articular clinical expression of tophaceous gout. The aim of this report is to describe the clinical characteristics and potential risk factors for the development of lobular panniculitis secondary to chronic tophaceous gout. Read More
Introduction: Rosai-Dorfman Disease (RDD), also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML), is a rare monocyte/macrophage proliferative disorder of varied biological behavior. Although cutaneous and lymph node involvement are relatively well-described, intrathoracic manifestations of RDD have only occasionally been reported.
Methods: We conducted a retrospective computer-assisted search of the Mayo Clinic record from 1976 to 2005 for patients with histopathologic evidence of RDD on organ biopsy. Read More
Purpose Of Review: There is an increasing interest in improving the understanding of pathophysiology, outcome measures, and therapies of rheumatic skin disease. Increasingly, studies are using the skin as a primary endpoint for evaluating therapies. This will review the current state of the art for the most common rheumatic skin diseases. Read More
Objective: To investigate the contribution of inherited and acquired thrombophilic defects to the clinical manifestations of mixed cryoglobulinaemia vasculitis.
Methods: The following thrombophilic defects were investigated in 64 consecutive patients with HCV-associated mixed cryoglobulinaemia: aPLs, lupus anti-coagulant, homocysteinaemia, protein C and protein S concentrations, activated protein C resistance, plasminogen activator inhibitor-1 4G4G and 5G5G genotypes, and the presence of mutations of factor V (Leiden and H1299R), of prothrombin (G20210A) and of methyl tetrahydrofolate reductase (C677T and A1298C). Additional variables were demographic data, duration of the disease, cryocrit level and vascular risk factors (diabetes, hypertension, hypercholesterolaemia and smoking habit). Read More
Our objective was to characterize clinical features, laboratory findings, concomitant autoimmune diseases, and smoking habits of lupus erythematosus subgroups in genetically homogeneous patients from two Dermatology Departments of Finnish University hospitals. One hundred and seventy eight discoid lupus erythematosus, 55 subacute cutaneous lupus erythematosus, and 77 systemic lupus erythematosus patients were enrolled using patients' charts from institutional database (1995-2006) and during routine control visits. Clustering analysis was performed to reveal natural groupings. Read More
Background And Aim: Hepatitis C virus (HCV) causes liver diseases and extrahepatic manifestations, and also contributes to insulin resistance and type 2 diabetes mellitus (DM). The aims of the present study were to examine the incidence of extrahepatic manifestations including lichen planus in HCV-infected patients and to evaluate the relationship between lichen planus and insulin resistance.
Methods: Of 9396 patients with liver diseases presenting to the study hospital, 87 patients (mean age 60. Read More
Palmar erythema (PE), an often overlooked physical finding, is due to several physiologic or systemic pathologic states. PE can exist as a primary physiologic finding or as a secondary marker of systemic pathology. Primary or physiologic PE can be due to heredity, occurs in at least 30% of pregnant women as a result of associated alterations in the function of the skin and its microvasculature, or may be a diagnosis of exclusion (i. Read More
Background: At the beginning the antiphospholipid antibodies syndrome was associated with systemic lupus erythematosus. But since 1988 it has become a sole entity. Its current definition is based on the criteria established in 1999 by Sapporo and consists of associating the clinical criteria of thrombosis of arteries or peripheral veins and of miscarriage of pregnancy with the biological criteria. Read More
Subacute cutaneous lupus erythematosus (SCLE) represents a widespread, photosensitive, nonscarring, nonindurated form of lupus erythematosus (LE)-specific skin disease. SCLE lesions are associated with a distinctive immunogenetic background including the production of Ro/SS-A autoantibodies. Individuals who have SCLE skin lesions as a component of their presenting illnesses represent a distinctive subset (subphenotype) of LE that enjoys a good prognosis with respect to life-threatening systemic manifestations of LE. Read More
Background: Cigarette smoking is the single biggest preventable cause of death and disability in developed countries and is a significant public health concern. While known to be strongly associated with a number of cardiovascular and pulmonary diseases and cancers, smoking also leads to a variety of cutaneous manifestations.
Objective: This article reviews the effects of cigarette smoking on the skin and its appendages. Read More
Objective: Some of the obsessive-compulsive disorder (OCD) symptoms can be elicited in rats by the administration of quinpirole (D2/D3 dopaminergic agonist). Nicotine administration blocked some aspect of checking behavior in that model. The main goal of this study was to determine if the clinical manifestations of OCD non smoking patients change with the administration of transdermal nicotine patches. Read More
Heavy smokers are at risk of aggravating several cutaneous diseases. The main adverse effects of cigarette smoking on the skin are associated with psoriasis, with squamous cell carcinoma and with the poorer outcome of malignant melanoma. One of the main concerns to smokers is the well-documented effect of smoking on premature face aging due to excessive wrinkling, which may follow enhanced elastase activity, and the degradation of elastin in the dermis. Read More
Background: The HIV/AIDS epidemic in Cambodia has become a major problem in the last 7-8 years, mainly because in this formerly war-stricken country the socioeconomic situation is only slowly improving. Since only very few studies have been published to date on the oral health status of Cambodian HIV/AIDS patients, it was the purpose of the present investigation to study oral manifestations in Cambodian patients with HIV disease.
Methods: One hundred one Cambodian patients with HIV infection or AIDS were examined for the presence of oral manifestations in one medical center in Phomh Penh, Cambodia. Read More
We report 2 patients with the association of Crohn's disease and acne inversa (= hidradenitis suppurativa). A 50-year-old woman with a 10-year-history of Crohn's disease developed suppurative nodules and abscesses in the genitoanal region. Over several years these lesions were hidden from everybody including the family physician. Read More
Background: The cholesterol embolism syndrome is a multisystemic disease resulting from cholesterol crystal embolization to many organs including skin, kidney and CNS. Vascular procedures and anticoagulation have been identified as triggering factors.
Patients And Methods: Sixteen patients were prospectively reviewed diagnosed of cholesterol embolism syndrome from 1991 to 1998. Read More
Patients with polycethemia vera (PV) or essential thrombocythemia (ET) are at increased risk of arterial and venous thromboembolic events. Arterial ischemic complications occur in 24 to 43% of these patients, particularly those with cardiovascular risk factors (especially cigarette smoking). Non-atheromatous arterial thrombosis concerns all large and medium-sized vessels, particularly cerebral, limb, coronary and digestive arteries. Read More
Chronic liver disease is often accompanied by hypoxaemia. We investigated the clinical factors that were related to the arterial oxygen tension (PaO2) in 40 women, all non-smokers with chronic liver disease. They were positive for hepatitis C virus (HCV) antibody and had no evidence of cardiopulmonary disease. Read More
We have studied a 38-year-old man with a prior diagnosis of Holt-Oram syndrome, who presented with diabetes mellitus. He had recently taken prednisone for idiopathic interstitial lung disease and trimethoprim-sulfamethoxazole for sinusitis. Thrombocytopenia progressed to pancytopenia. Read More
Cigarette smoking is strongly linked to serious internal diseases such as cancer, cardiovascular disease, and lung disease. However, the external manifestations and consequences of smoking are relatively unknown. Although generally less ominous, the cutaneous manifestations of smoking may be associated with significant morbidity. Read More
The authors report the case of a 60-year-old white man with a previous history of pulmonary tuberculosis, smoking habits, hypertension, intermittent claudication and erythromelalgia, admitted to our ward with an ischemic cerebral event. Initial laboratory evaluation documented thrombocytosis (platelet-950000/mm3) and discrete anemia. Additional studies confirmed the diagnosis of essential thrombocythemia, meeting all the criteria proposed by the Polycythemia Vera Study Group in 1986, after exclusion of the possible causes of reactive thrombocytosis. Read More
Diseases caused by occupational exposure to sensitizing metals including platinum (Pt), rhodium (Rh), nickel (Ni), chromium (Cr), cobalt (Co), gold (Au), mercury (Hg), zirconium (Zr) and beryllium (Be) are reviewed. Allergic reactions induced by the metals are described according to the classification by Coombs and Gell. Metals with unproven sensitizing potential are not discussed if reports on these are either very rare or devoid of convincing evidence for allergic involvement. Read More
The authors describe three young female patients with generalized livedo racemosa and cerebral infarction. The radiologic and biopsy findings point to a widespread vasculopathy, involving small and mid-sized arteries. The histologic examination of the temporal artery biopsy in two patients gives additional evidence for a chronic endarteritis as possible underlying cause of this disease entity. Read More