Scleroderma

• Systemic sclerosis
• Localized scleroderma
• CREST syndrome

• 701.0 Circumscribed scleroderma
• 710.1 Systemic sclerosis

• 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

• L94.0 Localized scleroderma [morphea]
• L94.3 Sclerodactyly
• M34.0 Progressive systemic sclerosis
• M34.1 CR(E)ST syndrome
• M34.9 Systemic sclerosis, unspecified

• 4D: Impaired joint mobility, motor function, muscle performance, and range of motion associated with Connective Tissues Dysfunction1
• 4E: Impaired joint mobility, motor function, muscle performance, and range of motion associated with localized inflammation2
• 7B: Impaired integumentary integrity associated with superficial skin involvement3
• 7E: Impaired Integumentary Integrity Associated With Skin Involvement Extending Into Fascia, Muscle, or Bone and Scar Formation4

Description

• 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

Essentials of Diagnosis

• 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

General Considerations

• 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

Demographics

• 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 Caucasians
• More severe in African and Native Americans

Signs and Symptoms

• Raynaud’s phenomenon
• Gastroesophageal reflux disease (GERD)
• Skin changes: change in color or thickening (sclerodactyly) usually on ...