While assessing the skin of a client, the primary healthcare provider identifies actinic keratosis. Which clinical findings support this conclusion? Show
Erythematous, barely elevated plaques Elevated, dry, hyperkeratotic scaly papules Variegated colors of tan, brown, black within a single mole Thin, scaly, erythematosus plaque without invasion into the dermis Systemic SclerodermaNORD gratefully acknowledges Etienne Leveille, MD Candidate, McGill University School of Medicine, and John Varga, MD, John and Nancy Hughes Distinguished Professor in the Division of Rheumatology; Director of the Scleroderma Program, Northwestern University Feinberg School of Medicine, for assistance in the preparation of this report. Synonyms of Systemic Scleroderma
Subdivisions of Systemic Scleroderma
General DiscussionSystemic scleroderma is a disease characterized by rapid growth of fibrous (connective) tissue that leads to scarring of skin and internal organs. Approximately one in 10,000 individuals is affected. It is more common in women and most often develops around age 30 to 50. Systemic scleroderma can affect almost any organ in the body, and there is a large variability of symptoms among affected individuals. One of the most common and earliest manifestations of the disease is Raynaud phenomenon, which involves blood vessel spasms (vasospasms) induced by cold temperature or stress. This can lead to temporary finger discoloration, numbness and pain and is also associated with the development of finger ulcers. Of note, Raynaud phenomenon also commonly occurs in healthy individuals. Other manifestations of systemic scleroderma include muscle and joint pain, skin tightening, and dilated blood vessels that can be seen through the skin (telangiectasias). Scarring of internal organs can also lead to gastrointestinal, pulmonary, cardiac, and renal disease. Although systemic scleroderma cannot be cured, many of the symptoms can be treated. A timely diagnosis is important to ensure appropriate management of the disease and associated complications. Signs & SymptomsSystemic scleroderma can affect multiple organ systems and therefore lead to numerous symptoms and complications. The symptoms present, their severity, the rate of progression of the disease, the response to treatment, and overall survival vary widely depending on the affected individual. In most cases, people start to develop symptoms between age 30 and 50. Symptoms related to the different organs involved are described below. Skin Nerves, muscles, and joints Lungs Gastrointestinal tract Heart Kidneys Others Clinical subsets CausesAlthough the exact cause and disease mechanism of systemic scleroderma are not known, it is thought to occur in genetically predisposed individuals following a trigger, possibly exposure to virus or toxins. Systemic scleroderma is an autoimmune disease, which means that it involves a dysregulated immune system that attacks the affected individual’s own body. The first event to occur is possibly injury to small blood vessels (microvascular injury). In healthy individuals, response to injury leads to recruitment of inflammatory mediators that facilitate repair. In individuals with systemic scleroderma, microvascular injury leads to disproportionate inflammatory mediator recruitment and excessive fibrous tissue deposition. This fibrous tissue can replace healthy tissue and lead to symptoms of systemic scleroderma such as skin thickening, internal organ scarring and associated complications. Affected PopulationsSystemic scleroderma affects between 38 and 341 individuals per million throughout the world (prevalence) and develops in 8 to 56 individuals per million each year (incidence). It is more common in populations from southern Europe, North America and Australia, and less common in populations from northern Europe and Japan. The disease most often starts to manifest in the fifth decade of life (age of onset). Although it most commonly occurs in women, men tend to have more severe disease. African American individuals tend to have a lower age of onset, higher rates of the diffuse cutaneous subtype, and overall more severe disease. DiagnosisSystemic scleroderma is a complex disorder that can be difficult to diagnose. In most cases, the diagnosis starts with a complete patient history and physical examination. If systemic scleroderma is suspected, laboratories test can be ordered. Notably, certain antibodies reacting against components of an individual’s body (autoantibodies) can be identified in systemic scleroderma. Antinuclear antibodies are present in approximately 95% of individuals. However, they are also present in other autoimmune conditions and in healthy individuals. Antitopoisomerase I (anti-Scl-70) antibodies and anti-RNA polymerase III antibodies are associated with dcSSc, while anticentromere antibodies can be seen in lcSSc. If antibodies titers are indicative of systemic scleroderma, specific tests will be performed to confirm the diagnosis and evaluate for internal organ involvement. Pulmonary function tests (PFTs) are breathing tests used to assess the lungs capacity to move air and diffuse it to the blood. They are often complemented by a computed tomography (CT) scan of the chest to visually assess the structure of the lungs. The heart and pulmonary arteries can be imaged with cardiac echocardiography, which uses ultrasound waves to reconstruct and visualize anatomical structures. Renal function can notably be evaluated by measuring creatinine levels in the blood and by urine analysis. Further testing might be indicated depending on the symptoms and characteristics of the affected individual. Standard TherapiesTreatment & Management As there is no cure for systemic scleroderma, management of the disease is mostly centered on symptom control and screening to detect and better control complications. Patients usually require long-term regular follow-up with numerous medical specialists. As systemic scleroderma is an autoimmune disease, medications that suppress the immune system (immunosuppressants) can be used, especially in severe cases with diffuse skin involvement, interstitial lung disease, inflammation of the heart muscle (myocarditis) and severe muscle or joint inflammation. Immunosuppressants used in systemic scleroderma include methotrexate, mycophenolate mofetil (MMF), and azathioprine. Glucocorticoids such as prednisone are also occasionally used in some patients, but their use is generally avoided if possible due to the risk of side effects. Skin, muscles, joints, and nerve Lungs Gastrointestinal tract Heart Kidneys Others Investigational Therapies
As the mechanisms responsible for the development and progression of the disease are uncovered, new medications or medications already on the market for other diseases are tested in clinical trials. Example of targeted therapies being investigated include rilonacept and tocilizumab, which respectively target IL-1 and IL-6, two inflammatory mediators thought to be important in systemic scleroderma. Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site. For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office: Toll-free: (800) 411-1222 Some current clinical trials also are posted on the following page on the NORD website: For information about clinical trials sponsored by private sources, contact: For information about clinical trials conducted in Europe,
contact: NORD Member Organizations
Other Organizations
ReferencesINTERNET Denton CP,
Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults, UpToDate. Last updated: Nov 20, 2019. Denton CP, Pathogenesis of systemic sclerosis (scleroderma), UpToDate. Last updated: Jun 14, 2019. https://www.uptodate.com/contents/pathogenesis-of-systemic-sclerosis-scleroderma Accessed March 10, 2020. Greidinger EL, Mixed Connective Tissue Disease, National Organization for Rare Disorders. Last updated: 2017. https://rarediseases.org/rare-diseases/mixed-connective-tissue-disease-mctd/ Accessed March 10, 2020. Kaye-Barrett SA, Denton CP, Gastrointestinal manifestations of systemic sclerosis (scleroderma), UpToDate. Last updated: Sep 25, 2018. King TE, Approach to the adult with interstitial lung disease: Clinical evaluation, UpToDate. Last updated: Aug 06, 2019. https://www.uptodate.com/contents/approach-to-the-adult-with-interstitial-lung-disease-clinical-evaluation Accessed March 10, 2020. Mukherjee M, Shah S, Varga J, Cardiac manifestations of systemic sclerosis (scleroderma), UpToDate. Last updated: Oct 29, 2019. Bundra K, Oddis CV, Dermatomyositis, National Organization for Rare Disorders. Last updated: 2018. https://rarediseases.org/rare-diseases/dermatomyositis/ Accessed March 10, 2020. Bundra K, Oddis CV, Polymyositis and Necrotizing Myopathy, National Organization for Rare Disorders. Last updated: 2019. https://rarediseases.org/rare-diseases/polymyositis/ Accessed March 10, 2020. Paik JJ, Varga J, Neuromuscular manifestations of systemic sclerosis (scleroderma), UpToDate. Last updated: Feb 05, 2019. Rubin LJ, Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults, UpToDate. Last updated: May 17, 2019. Sanchorawala V, Amyloidosis, National Organization for Rare Disorders. Last updated: 2018. https://rarediseases.org/rare-diseases/amyloidosis/ Accessed March 10, 2020. Systemic scleroderma, Genetic and Rare Diseases Information Center. Last updated: 26 Apr 2018. https://rarediseases.info.nih.gov/diseases/9748/systemic-sclerosis Accessed March 10, 2020. Varga J, Clinical manifestations and
diagnosis of systemic sclerosis (scleroderma) in adults, UpToDate. Last updated: Jan 03, 2020. Varga J, Clinical manifestations, evaluation, and diagnosis of interstitial lung disease in systemic sclerosis (scleroderma), UpToDate. Last updated: Feb 07, 2019. https://www.uptodate.com/contents/clinical-manifestations-evaluation-and-diagnosis-of-interstitial-lung-disease-in-systemic-sclerosis-scleroderma Accessed March 10, 2020. Varga J, Overview of pulmonary complications of systemic sclerosis (scleroderma), UpToDate. Last updated: Aug 19, 2019. https://www.uptodate.com/contents/overview-of-pulmonary-complications-of-systemic-sclerosis-scleroderma Accessed March 10, 2020. Varga J, Fenves AZ, Renal disease in systemic sclerosis (scleroderma), including scleroderma renal crisis, UpToDate. Last updated: Feb 12, 2019. https://www.uptodate.com/contents/renal-disease-in-systemic-sclerosis-scleroderma-including-scleroderma-renal-crisis Accessed March 10, 2020. Varga J, Montesi S, Treatment and prognosis of interstitial lung disease in systemic sclerosis (scleroderma), UpToDate. Last updated: Oct 08, 2019. https://www.uptodate.com/contents/treatment-and-prognosis-of-interstitial-lung-disease-in-systemic-sclerosis-scleroderma Accessed March 10, 2020. Varga J, Steen V, Pulmonary arterial hypertension in systemic sclerosis (scleroderma): Definition, classification, risk factors, and screening, UpToDate. Last updated: Feb 06, 2019. https://www.uptodate.com/contents/pulmonary-arterial-hypertension-in-systemic-sclerosis-scleroderma-definition-classification-risk-factors-and-screening Accessed March 10, 2020. Varga J, Steen V, Hassoun P, Pulmonary arterial hypertension in systemic sclerosis (scleroderma): Treatment and prognosis, UpToDate. Last updated: Jan 10, 2020. https://www.uptodate.com/contents/pulmonary-arterial-hypertension-in-systemic-sclerosis-scleroderma-treatment-and-prognosis Accessed March 10, 2020. Wallace DJ, Patient education: Systemic lupus erythematosus (Beyond the Basics), UpToDate. Last updated: Dec 28, 2019. https://www.uptodate.com/contents/systemic-lupus-erythematosus-beyond-the-basics Accessed March 10, 2020. Wigley FM, Clinical manifestations and diagnosis of Raynaud phenomenon, UpToDate. Last updated: Oct 08, 2019. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-raynaud-phenomenon Accessed March 10, 2020. JOURNAL ARTICLES Denton CP, Khanna D. Systemic sclerosis. Lancet 2017;390:1685-99. Allanore Y, Simms R, Distler O, et al. Systemic sclerosis. Nat Rev Dis Primers 2015;1:15002. Barnes J, Mayes MD. Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol 2012;24:165-70. The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional. The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright. National Organization for Rare Disorders (NORD) Which common complications could be seen in a client who has undergone surgery for bags under the eyes?These include discomfort, bruising and swelling of the eyelid, increased intraocular pressure, and allergic reaction to the steroid or antibiotic drop. These complications are monitored over time following surgery.
Which skin color alteration may be observed in a client diagnosed with methemoglobinemia?Symptoms are proportional to the methemoglobin level and include skin color changes (cyanosis with blue or grayish pigmentation) and blood color changes (brown or chocolate color).
Which organism infestation is diagnosed with the help of the mineral oil test?Diagnosis and Tests
Most cases of scabies can be confirmed just by looking closely at the skin. Your healthcare provider may also apply mineral oil to the rash and use a scalpel to get a small sample of skin (scraping). The sample is placed under a microscope and examined for mites and mite eggs.
Which technology would the nurse use to reduce chronic ulcers by removing fluids from the wound?A wound vacuum system may help your wound heal more quickly by: Draining excess fluid from the wound. Reducing swelling. Reducing bacteria in the wound.
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