Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.

Background: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc).

Methods: In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema.

Results: Rate of CPFE in SSc patients with CT scan was 3.

Smoking reduces the efficacy of belimumab in mucocutaneous lupus.

Objectives: Recently, we demonstrated a negative impact of smoking on belimumab efficacy in patients with systemic lupus erythematosus (SLE). Here, we particularly investigated clinical effects of belimumab and a potential impact of smoking in mucocutaneous and articular SLE.

Methods: We surveyed 62 SLE patients treated between 2011 and 2017.

Brief Report: Smoking in Systemic Sclerosis: A Longitudinal European Scleroderma Trials and Research Group Study.

Objective: Data on the role of tobacco exposure in systemic sclerosis (SSc; scleroderma) severity and progression are scarce. We aimed to assess the effects of smoking on the evolution of pulmonary and skin manifestations, based on the European Scleroderma Trials and Research group database.

Methods: Adult SSc patients with data on smoking history and a 12-24-month follow-up visit were included.

Disease severity and prophylactic measures in patients with cutaneous lupus erythematosus: results of a worldwide questionnaire-based study.

Introduction: Due to a wide array of dermatologic manifestations, assessment of disease severity in cutaneous lupus erythematosus (CLE) remains challenging. Given a need for some standardization in this field, we conducted a worldwide questionnaire-based study among physicians experienced in CLE management.

Aim: We asked about CLE assessment, their prophylactic measures advised to patients, and treatment recommendations.

Atmospheric skin aging-Contributors and inhibitors.

Cutaneous aging is a complex biological process consisting of 2 elements: intrinsic aging, which is primarily determined by genetics, and extrinsic aging, which is largely caused by atmospheric factors, such as exposure to sunlight and air pollution, and lifestyle choices, such as diet and smoking. The role of the solar spectrum, comprised of ultraviolet light, specifically UVB (290-320 nm) and UVA (320-400) in causing skin damage, including skin cancers, has been well documented. In recent years, the contribution of visible light (400-700 nm) and infrared radiation (above 800 nm) in causing skin damage, similar to the photodamage caused by UV light, is also being elucidated.

Hidradenitis Suppurativa and Crohn's Disease: A Case Series.

Hidradenitis Suppurativa (HS) is characterized by chronic recurrent abscesses, nodules and draining sinus tracts with scar formation. Cutaneous Crohn's Disease (CD) may also present similarly. We wished to identify and describe an Irish cohort with combined HS and CD, with a view to a better recognition of clinical manifestations and understanding of the pathophysiology underlying these two overlapping conditions.

Scalp metastasis as an initial presentation of lung adenocarcinoma : A case report and literature review.

Introduction: Cutaneous metastasis from primary visceral malignancy is a relatively uncommon clinical entity, with a reported incidence of 0.22%-10% among various series. However, the presence of cutaneous metastasis as the first sign of a clinically silent visceral cancer is exceedingly rare.

Transient left ventricular dysfunction due to coronary spasm after spinal anesthesia with bupivacaine - a case report.

Bupivacaine is a long-acting local anesthetic (LA) used for cutaneous infiltration, peripheral nerve blocks, epidural and spinal anesthesia. However, its application may result in cardiovascular complications such as: hypotension, bradycardia, cardiac arrest and toxic myocardial injury. The authors describe a 53-year-old male with a history of cigarette smoking, admitted for an elective inguinal hernia surgery.

Cutaneous and Mucosal Manifestations of Sjögren's Syndrome.

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.

Generalized Discoid Lupus Erythematosus as the Presenting Sign of Small Cell Lung Carcinoma.

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.

Cocaine-induced vasculitis with cutaneous manifestation: A recurrent episode after 2 years.

Cocaine is a popular recreational drug in the United States, and up to 70% of the seized cocaine contains levamisole which is an antihelminthic that can cause cutaneous vasculitis with necrosis and positive antineutrophil cytoplasmic antibodies (ANCAs). Here, we report a unique case of recurrent cocaine-induced vasculitis in a patient who smokes cocaine for more than 20 years. A 38-year-old woman complained of painful erythematous rash in her right arm and right thigh which appeared some hours after smoking cocaine.

Hepatic porphyria: A narrative review.

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).

The relationship between vascular biomarkers and disease characteristics in systemic sclerosis: elevated MCP-1 is predominantly associated with fibrotic manifestations.

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.

Hydradenitis suppurativa and inflammatory bowel disease: An unusual, but existing association.

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.

Dramatic course of osteomyelitis in a patient treated with immediately placed dental implants suffering from uncontrolled diabetes: A case report.

Purpose: To report a rare and dramatic complication following immediate dental implant placement in a heavy smoker, who had a delayed diagnosis of uncontrolled diabetes.

Materials And Methods: In this case report we present the dramatic course of a 64-year old female patient treated with five immediate post-extractive dental implants in the mandible, who developed osteomyelitis, which manifested initially as local peri-implant inflammation and progressed into a spontaneous jaw fracture, despite repeated surgical interventions and antibiotic courses over a 3-year period, until diabetes was diagnosed.

Results: A symptom-free status could be achieved only after partial mandibulectomy, treatment of diabetes and reconstruction with a microvascular fibula free flap.

[Cutaneous lupus erythematosus].

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).

Thyroid follicular carcinoma presenting as metastatic skin nodules.

Background: Follicular thyroid cancer (FTC) metastasizes most commonly to the lungs and non-cranial bones. Skull and skin are uncommon sites and usually manifest well after the diagnosis of primary malignancy. Metastasis to skull and skin as the presenting feature of FTC is infrequently reported in the literature.

PSEUDOXANTHOMA ELASTICUM: DIAGNOSTIC FEATURES, CLASSIFICATION, AND TREATMENT OPTIONS.

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 gene.

Extraintestinal manifestations of inflammatory bowel disease and the influence of smoking.

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.

Environmental factors in benign migratory glossitis and psoriasis: retrospective study of the association of emotional stress and alcohol and tobacco consumption with benign migratory glossitis and cutaneous psoriasis.

Background: The association between benign migratory glossitis (BMG) and psoriasis (PS) has been reported in the literature.

Objective: This study aimed to determinate the environmental factors related to BMG and PS and to investigate their interactions.

Methods: The study population included 129 patients with PS, 399 patients with BMG and a control group (CG) of 5472 individuals with neither PS nor BMG.

Scalp metastasis as the first sign of small-cell lung cancer: management and literature review.

Cutaneous metastasis from primary visceral malignancy is a relatively uncommon clinical entity, with a reported incidence ranging from 0.22% to 10% among various series. However, the presence of cutaneous metastasis as the first sign of a clinically silent visceral cancer is exceedingly rare.

A longitudinal study of ankle brachial pressure indices in a cohort of patients with systemic sclerosis.

Objectives: Digital ischaemia, often progressing beyond RP to digital ulceration and sometimes even gangrene, is the most common vascular manifestation of SSc. Both microvascular and macrovascular disease can contribute and coexistence of microvascular and macrovascular (proximal vessel) disease in patients with SSc is potentially limb threatening. The aims of this study were to examine the change over time in the ankle brachial pressure index (ABPI) in a cohort of patients with SSc and to examine whether age, gender, smoking status, disease duration, disease subtype and ACA are associated with ABPI.

Characteristics, prognosis, and outcomes of cutaneous ischemia and gangrene in systemic necrotizing vasculitides: a retrospective multicenter study.

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.

Predictive factors for erythema nodosum and pyoderma gangrenosum in inflammatory bowel disease.

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.

Psoriasis outcome measures: a report from the GRAPPA 2012 annual meeting.

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.