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August 18, 2008

Coding for Scleroderma
For The Record
Vol. 20 No. 17 P. 28

Scleroderma is a chronic arthritic condition that results in hardening and tightening of the skin and connective tissues due to an overproduction and accumulation of collagen in the body tissues. Literally translated to mean “hard skin,” scleroderma begins as a few dry patches of skin on the hands or face that eventually get thicker and harder; it may also affect blood vessels and other internal organs. The condition, which is not contagious, infectious, or cancerous, may range in seriousness from mild to life threatening. There is currently no cure for scleroderma, and the autoimmune disorder may be fatal if left untreated.

Signs and Symptoms
Some of the common symptoms of scleroderma include thick, hardened skin; a loss of skin elasticity and a shiny appearance; numbness, pain, or color changes in fingers, toes, cheeks, nose, or ears brought on by Raynaud’s phenomenon (digital cyanosis brought on by cold or emotional distress); stiffness or pain in joints; curling of fingers; digestive problems such as poor absorption of nutrients or delayed movement of food; sores over joints; and puffy hands and feet.

Types of Scleroderma
Scleroderma may be categorized as localized or systemic. Localized scleroderma (ICD-9-CM code 701.0) may be limited to the skin and deep tissues below the skin. Two subcategories of localized scleroderma (both included in code 701.0) are as follows:

• Morphea — oval-shaped, thick patches appear on the skin, which are white in the middle with a purple border. This form of scleroderma most likely occurs on the torso but may also appear on the arms, legs, or forehead.

• Linear scleroderma — results in bands or streaks of hardened skin on one or both of the arms or legs or on the forehead.

Systemic scleroderma (710.1) affects not only the skin but also blood vessels and major organs. Sometimes called systemic sclerosis, the subcategories (all classified to code 710.1) include the following:

• Diffuse cutaneous systemic sclerosis — affects skin of the fingers, hands, arms, legs, face, neck, and trunk. It also affects internal organs such as the lungs, heart, kidneys, and esophagus.

• Limited cutaneous systemic sclerosis — involves skin of the fingers, lower arms and legs, face, and neck. It may also be documented as CREST or CRST syndrome.

• Sine scleroderma — similar to either limited or diffuse scleroderma, except it doesn’t affect the skin.

Complications of Scleroderma
Systemic scleroderma may affect internal organs and cause additional problems. Assign the code for systemic scleroderma (710.1) first, followed by an additional code to identify manifestations. Gastrointestinal complications may include reduction of absorption of nutrients and decreased movement within the intestine. Lung complications may include scarring of lung tissue and result in pulmonary hypertension. Kidney complications involve elevated blood pressure and increased level of protein in the urine and may result in renal crisis or kidney failure. Heart complications include scarring of the heart tissue and may result in arrhythmia, congestive heart failure, or pericarditis.

Scleroderma can look like other diseases, so it is often difficult to diagnose. The physician will review the patient’s history and perform a complete physical examination, including evaluating the skin to check for thickened and hardened areas. The physician will also check for changes in connective tissue beneath the skin. Blood tests may be ordered to check for elevated blood levels of certain antibodies. The physician may also choose to perform a skin biopsy.

Although there is no known cure and treatment will not stop the overproduction of collagen, medications will treat symptoms and complications of scleroderma. Common medications include minocycline (Minocin, Dynacin); calcium channel blockers; alpha blockers; angiotensin-converting enzyme inhibitors; angiotensin II receptor blockers; low-dose enteric-coated aspirin; nonsteroidal anti-inflammatory drugs; low-dose corticosteroids; disease-modifying antirheumatic drugs such as hydroxychloroquine (Plaquenil), penicillamine (Cuprimine, Depen), and methotrexate (Rheumatrex, Trexall); and cyclophosphamide (Cytoxan).

Coding and sequencing for scleroderma are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding.

— This information was prepared by Audrey Howard, RHIA, of 3M Consulting Services. 3M Consulting Services is a business of 3M Health Information Systems, a supplier of coding and classification systems to nearly 5,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payers as the result of the misuse of this coding information. More information about 3M Health Information Systems is available at www.3mhis.com or by calling 800-367-2447.