Localized scleroderma (involving only the skin) Morphea is characterized by firm, hardened patches of skin. CREST is a form of scleroderma that results in calcium deposits in the skin, Raynaud’s phenomenon, esophageal involvement, swollen fingers with tight skin, and reddened/discolored skin from by blood vessels. The PSS classification is made on the basis of the involvement of both skin and organs. Diffuse myocardial fibrosis or cardiac conduction abnormalities occur. Digital ulcers are common, especially on the fingertips, overlying the finger joints, or over calcinotic nodules. The "C" stands for calcinosis, where calcium deposits form under the skin on the fingers or other areas of the body. Systemic sclerosis–like syndromes have been associated with vinyl chloride, Pathophysiology involves vascular damage and activation of fibroblasts; collagen and other extracellular proteins in various tissues are overproduced.In systemic sclerosis, the skin develops more compact collagen fibers in the reticular dermis, epidermal thinning, loss of rete pegs (epithelial extensions that project into the underlying connective tissue), and atrophy of dermal appendages. Hypomotility of the small bowel causes bacterial overgrowth that can lead to malabsorption. Diagnosis is clinical, but laboratory tests support the diagnosis and aid in prognostication. Systemic Sclerosis of the Hand Systemic Sclerosis of the Chest and Shoulders Symptoms of CREST syndrome. Friction rubs may develop over the joints, tendon sheaths, and large bursae.Esophageal dysfunction is the most frequent visceral disturbance and occurs in most patients. Leader5 suggests a physical therapy technique used to increase the mouth opening over time: Lubricate the mouth, have the patient cross arms at chest height, with palms facing down, and use the thumbs to stretch the corners of the mouth for several minutes. Renal transplantation is a feasible option in patients who develop end-stage renal disease.Some experts encourage periodic screening for pulmonary hypertension with pulmonary function testing and/or echocardiography every 1 to 2 years, depending on symptoms.Key findings in systemic sclerosis include skin and joint changes, Raynaud phenomenon, and esophageal changes, but life-threatening effects may involve organs such as the lungs, heart, or kidneys.Consider the diagnosis if patients have Raynaud phenomenon, typical musculoskeletal or skin manifestations, or unexplained dysphagia, malabsorption, interstitial lung disease and pulmonary fibrosis, pulmonary hypertension, cardiomyopathies, or conduction disturbances.Test for ANA, Scl-70 (topoisomerase I), and anticentromere antibodies.Because there is no clear disease-modifying therapy, direct treatment at involved organs.Merck and Co., Inc., Kenilworth, NJ, USA (known as MSD outside the US and Canada) is a global healthcare leader working to help the world be well. On physical examination, there is tenderness to palpation of the arm and a mass is noted. There is no history of trauma to the arm. Depending upon the particular patient, the pigmentation may be slight or more extensive.Sign up for Registered Dental Hygienist (RDH) Magazine eNewsletters© 2020 Endeavor Business Media, LLC.

Diagnosis of systemic sclerosis can be obvious in patients with combinations of classic manifestations, such as Raynaud phenomenon (with abnormal nail-fold capillary findings), dysphagia, and tight skin. Click here for Patient Education View All Procedures > Click here for the Consumer Version CREST stands for its group of symptoms. This primarily affects the skin of the face, hands and feet (with possible involvement of other organs). Caries and periodontal involvement may be lessened with careful monitoring and patient care early in the disease process. In Figure 2, the skin and oral tissues are tight even with the aid of mirrors. Pericarditis with effusion or pleurisy can occur.

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Since it is summer, your attention is focused on her covered hands and long sleeve sweater. These are calcinosis, Raynaud syndrome, esophageal dysmotility, sclerodactyly, and telangiectasia.

Treatment involves behavior changes, medication (H2 blockers), and a surgical procedure (esophageal dilatation) in more severe cases. In the nail folds, capillary loops dilate and some microvascular loops are lost. CREST syndrome is an autoimmune disorder that is a localized variant of systemic scleroderma, a chronic connective tissue disease.

Flexor Tendon Sheaths of the Fingers Abnormal capillary and microvascular loops in the nails can be seen with an ophthalmoscope or dissecting microscope.This image shows shiny and thickened skin with effacement of normal markings secondary to tautness, called sclerodactyly.In this image, bound-down taut skin throughout the chest also extends over the shoulders bilaterally, leading to a loss of range of movement of the shoulders.In this photo, hardening and tightening of the skin has caused toes to curl in on themselves.Polyarthralgias or mild arthritis can be prominent.

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