Dermatomyositis
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Dermatomyositis
Pathology
Pediatric musculoskeletal conditions
Radial head subluxation (Nursemaid elbow)
Developmental dysplasia of the hip
Legg-Calve-Perthes disease
Slipped capital femoral epiphysis
Transient synovitis
Osgood-Schlatter disease (traction apophysitis)
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Spondylitis
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Bursitis
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Muscular disorders
Muscular dystrophy
Polymyositis
Dermatomyositis
Inclusion body myopathy
Polymyalgia rheumatica
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Sjogren syndrome
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Musculoskeletal system pathology review
Back pain: Pathology review
Rheumatoid arthritis and osteoarthritis: Pathology review
Seronegative and septic arthritis: Pathology review
Gout and pseudogout: Pathology review
Systemic lupus erythematosus (SLE): Pathology review
Scleroderma: Pathology review
Sjogren syndrome: Pathology review
Bone disorders: Pathology review
Bone tumors: Pathology review
Myalgias and myositis: Pathology review
Neuromuscular junction disorders: Pathology review
Muscular dystrophies and mitochondrial myopathies: Pathology review
Pediatric musculoskeletal disorders: Pathology review
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Dermatomyositis
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Dermatomyositis p. 221
autoantibody p. 113
Dermatomyositis/polymyositis p. 483
“Mechanic’s hands” in dermatomyositis p. 483
Polymyositis/dermatomyositis p. 483
Steroids
polymyositis/dermatomyositis p. 483
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Transcript
In dermatomyositis, “-itis” refers to inflammation, “myos-“ to the muscles and “dermato-“ to the skin, so dermatomyositis is an inflammatory disorder which involves both the skin and the muscles.
Dermatomyositis is considered to be an autoimmune disease, meaning that the immune system has gone rogue and started attacking its own muscles and skin.
Okay, normally, the cells of the immune system are always hanging around, ready and excited to spot and fight against anything foreign that could cause harm inside the body.
B- lymphocytes produce antibodies against a specific part of these foreign pathogens, called antigen.
The tips of these antibodies strongly binds to this antigen, while the base of the antibody, called the constant region, gets recognized by complement proteins.
These complement proteins are a group of small proteins made by the liver that work together.
One complement protein cuts or cleaves the next one, activating it and creating an enzymatic cascade.
This process gets started with C1, the first of the complement proteins, which binds to the Fc, or the constant region of two antibody attached to the pathogen.
C1 then cleaves C2 and C4.
Portions of the C2 and C4 binds to the antigen and form an enzymatic complex that cleaves C3 into two portions, C3a and C3b.
C3b joins the enzymatic complex and then the complex is able to cleave C5 into two portions, C5a and C5b portion.
C5a and C3a float off into the blood where they attract other cells of the immune system to the affected area.
Meanwhile, C5b, C6, C7, C8 and multiple C9 proteins, come together on the surface of the pathogen to form the membrane attack complex or MAC.
The MAC attacks pathogenic cells, such as bacteria, by creating a channel in the cell membrane.
Because cells have more solutes in them than the outside environment, water flows into the cell by the process of osmosis, and that causes the cell to swell up and burst, which is called cell lysis.
In dermatomyositis, immune cells confuse normal muscle and skin proteins with foreign antigens.
This process is called molecular mimicry because from the perspective of the immune cells, a host protein is mimicking a foreign or tumor protein.
Summary
Dermatomyositis (DM) is a rare autoimmune disease that leads to inflammation and damage of the muscles and skin. It is associated with complement system activation, and autoantibodies like ANA, anti-Mi-2 and, anti-Jo-1 which result in proximal muscle weakness and photosensitive skin rashes. DM presents with muscle weakness, which often becomes worse over time, as well as a distinctive skin rash.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
- "Clinical Immunology" Elsevier (2018)
- "Dermatomyositis" Clinics in Dermatology (2006)
- "Treatment of clinically amyopathic dermatomyositis in adults: a systematic review" British Journal of Dermatology (2016)
- "Treatment of clinically amyopathic dermatomyositis in adults: a systematic review" British Journal of Dermatology (2016)
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