Scleroderma: Pathology review
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Scleroderma: Pathology review
Pathology
Bone disorders
Osteomyelitis
Bone tumors
Chondrosarcoma
Osteochondroma
Achondroplasia
Arthrogryposis
Cleidocranial dysplasia
Club foot
Craniosynostosis
Flat feet
Genu valgum
Genu varum
Osteogenesis imperfecta
Pectus excavatum
Pigeon toe
Lordosis, kyphosis, and scoliosis
Osteomalacia and rickets
Osteopetrosis
Osteoporosis
Osteosclerosis
Paget disease of bone
Musculoskeletal injuries and trauma
Degenerative disc disease
Sciatica
Spinal disc herniation
Spondylolisthesis
Spondylolysis
Achilles tendon rupture
Anterior cruciate ligament injury
Iliotibial band syndrome
Meniscus tear
Patellar tendon rupture
Patellofemoral pain syndrome
Sprained ankle
Unhappy triad
Compartment syndrome
Rhabdomyolysis
Carpal tunnel syndrome
Erb-Duchenne palsy
Klumpke paralysis
Sciatica
Thoracic outlet syndrome
Ulnar claw
Winged scapula
Carpal tunnel syndrome
Dislocated shoulder
Erb-Duchenne palsy
Klumpke paralysis
Radial head subluxation (Nursemaid elbow)
Rotator cuff tear
Thoracic outlet syndrome
Ulnar claw
Winged scapula
Musculoskeletal system pathology review
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Bone disorders: Pathology review
Bone tumors: Pathology review
Gout and pseudogout: Pathology review
Muscular dystrophies and mitochondrial myopathies: Pathology review
Myalgias and myositis: Pathology review
Neuromuscular junction disorders: Pathology review
Pediatric musculoskeletal disorders: Pathology review
Rheumatoid arthritis and osteoarthritis: Pathology review
Scleroderma: Pathology review
Seronegative and septic arthritis: Pathology review
Sjogren syndrome: Pathology review
Systemic lupus erythematosus (SLE): Pathology review
Assessments
Scleroderma: Pathology review
USMLE® Step 1 questions
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Questions
USMLE® Step 1 style questions USMLE
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Which of the following findings is most likely to be seen in this patient?
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While doing your rounds, you see Rosa, a 35-year-old woman who has complained of puffy hands and feet for the past 4 months.
On examination, the skin on the limbs and trunk is stiff and shiny, with decreased markings.
Other important findings are sclerodactyly, Raynaud's phenomenon, and digital ulceration.
Pulmonary function tests were performed as well, and they showed a pattern suggestive of restrictive lung disease.
Then you see Haruki, a 65-year old who says that he noticed skin changes recently, stating that the wrinkles on his face have disappeared.
He also said that his acid reflux got worse in the past 6 months.
On examination, his hands show Raynaud's phenomenon and sclerodactyly.
The skin on his face and the arms below the elbow were tight, shiny, smooth, with no wrinkles.
Pulmonary function tests are normal.
Blood tests were performed in both cases, showing increased serum levels of anti-Scl 70 and and-RNA polymerase III antibodies in Rosa, and increased anti-centromere antibodies in Haruki.
Now, both seem to have scleroderma.
Scleroderma refers to systemic sclerosis, a rare autoimmune disorder in which normal tissue is replaced by thick, dense collagen.
It affects the skin, blood vessels and internal organs.
Now, there are two main types of scleroderma, diffuse cutaneous systemic scleroderma; and limited cutaneous systemic scleroderma, which was formerly called CREST syndrome.
The condition’s pathology is not completely understood, but it’s believed that some individuals have a genetic predisposition to scleroderma which is triggered by external factors.
These triggers include: viral infection by cyto-megalo-virus and parvovirus B19; exposure to silica dust, organic solvents, vinyl chloride; and medication like cocaine, bleomycin, and pentazocine.
Summary
Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease characterized by progressive fibrosis of skin and internal organs such as the gastrointestinal tract, lungs, heart, and kidneys. The exact cause is unknown but is associated with autoimmune processes that lead to excessive collagen production in individuals with a genetic predisposition. Scleroderma can be either limited or diffuse, with symptoms varying according to the organs involved. Treatment options involve immunosuppressants and medications to relieve symptoms and slow the progression of the disease.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Diagnosis and Classification of Systemic Sclerosis" Clinical Reviews in Allergy & Immunology (2010)
- "Cellular and molecular mechanisms in the pathophysiology of systemic sclerosis" Pathologie Biologie (2015)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Systemic sclerosis/scleroderma: a treatable multisystem disease" Am Fam Physician (2008)
- "Following the Molecular Pathways toward an Understanding of the Pathogenesis of Systemic Sclerosis" Annals of Internal Medicine (2004)
- "The'CREST'Syndrome" Archives of Internal Medicine (1979)
- "New therapeutic strategies for systemic sclerosis--a critical analysis of the literature" Clin Dev Immunol (2005)
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