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Condition/Disorder Synonyms

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  • Systemic sclerosis

  • Localized scleroderma

  • CREST syndrome

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ICD-9-CM Codes

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  • 701.0 Circumscribed scleroderma

  • 710.1 Systemic sclerosis

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Associated Physical Therapy Diagnoses

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  • 315.4 Developmental coordination disorder

  • 718.03 Articular cartilage disorder, forearm

  • 718.04 Articular cartilage disorder, hand

  • 718.07 Articular cartilage disorder, ankle and foot

  • 718.45 Contracture of joint, pelvic region and thigh

  • 719.39 Palindromic rheumatism involving multiple sites

  • 719.4 Pain in joint

  • 719.70 Difficulty in walking

  • 728.2 Muscular wasting and disuse atrophy

  • 728.89 Other disorders of muscle, ligament, and fascia

  • 729.1 Myalgia and myositis, unspecified

  • 729.9 Other disorders of soft tissue

  • 736.9 Acquired deformity of limb

  • 780.7 Malaise and fatigue

  • 781.2 Abnormality of gait

  • 782.3 Edema

  • 786.0 Dyspnea and respiratory abnormalities

  • 786.05 Shortness of breath

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ICD-10-CM Codes

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  • L94.0 Localized scleroderma [morphea]

  • L94.3 Sclerodactyly

  • M34.0 Progressive systemic sclerosis

  • M34.1 CR(E)ST syndrome

  • M34.9 Systemic sclerosis, unspecified

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Preferred Practice Patterns

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Key Features

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Description

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  • Autoimmune skin disorder

  • Chronic, commonly progressive connective tissue disease considered an autoimmune rheumatic disease

  • Skin hardening

  • Intense fibrosis

  • Finger sensitivity to cold

  • Decreased sweating

  • Multiple body system involvement

  • Onset often vague, misdiagnoses common

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Essentials of Diagnosis

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  • Etiology unknown

  • Systemic scleroderma

    • Prolonged history of Raynaud's phenomenon before presenting with swollen fingers, heartburn, shortness of breath

  • Localized scleroderma

    • Morphea: oval-shaped skin patches with purplish borders that may fade over time

    • Linear scleroderma: bands of hardened skin on extremities or forehead, usually on one side of body

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General Considerations

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  • May result in secondary problems indicating need for PT intervention depending on severity: aerobic capacity and muscle endurance impairment, sarcopenia, weakness, musculoskeletal problems, neuromuscular problems, weight loss

  • Because scleroderma frequently refers or causes pain in various body areas, individuals may be inappropriately referred to PT, such as when referred to low back, upper back, chest

  • History of heartburn or indigestion may be related to scleroderma or may indicate GI or cardiac problem

  • Individuals with scleroderma have twice the incidence of breast and bronchoalveolar cancer than rest of population

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Demographics

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  • Females-to-male ratio: 7:1

  • Systemic scleroderma more common in adults; localized scleroderma most common in children

  • Between 2 and 20 cases per million people

  • Can affect individuals of any age; most frequent onset between 25 to 55 years of age, average onset in 40s

  • Decreased incidence after age 60 years

  • Some indication of genetic familial tendency

  • More common in ...

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