Dermatomyositis: Difference between revisions
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Classically presents with a symmetrical proximal myopathy with associated dermatological changes including, dusky-red rash over the face, arms, hands, legs and other features. Other features include, dysphagia, myalgia, fever and weight loss<ref name=":0" /> | Classically presents with a symmetrical proximal myopathy with associated dermatological changes including, dusky-red rash over the face, arms, hands, legs and other features. Other features include, dysphagia, myalgia, fever and weight loss<ref name=":0" /> | ||
[[File:Rash.jpg|alt=|thumb|200x200px|Rash]] | [[File:Rash.jpg|alt=|thumb|200x200px|Rash]] | ||
== Complications '''Malignancy''' == | |||
There is a sixfold increased risk of malignancy in dermatomyositis. This risk appears to be highest in patients diagnosed with dermatomyositis after 45 years of age. Risk factors include: >60 years old; male; dysphagia; necrosis of the skin; cutaneous vasculitis; accelerated onset of disease; increased creatine kinase (CK) levels; increased ESR and C-reactive protein (CRP) levels.<ref name=":0" /> | |||
== Medications<ref name="kasper">Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL. Harrison's Manual of Medicine. 16th ed. McGraw-Hill, 2005</ref><ref name="Callen" /> == | |||
'''Corticosteroids:''' | '''Corticosteroids:''' | ||
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'''Calcium channel blocker: '''(gradual resolution of calcinosis) | '''Calcium channel blocker: '''(gradual resolution of calcinosis) | ||
*Diltiazem | *Diltiazem<ref name="kasper" /><ref name="Callen" /> | ||
== Diagnostic Tests/Lab Tests/Lab Values<ref name="koler" /><ref name="Hajj-ali" /> == | == Diagnostic Tests/Lab Tests/Lab Values<ref name="koler" /><ref name="Hajj-ali" /> == |
Revision as of 07:14, 23 January 2023
Original Editors Megan Sarason from Bellarmine University's Pathophysiology of Complex Patient Problems project.
Top Contributors - Megan Sarason, Admin, Lucinda hampton, Elaine Lonnemann, Kim Jackson, Khloud Shreif, 127.0.0.1, Wendy Walker and WikiSysop
Introduction[edit | edit source]
Dermatomyositis is an autoimmune inflammatory myositis, possibly related condition to polymyositis.[1] It is characterized by inflammatory and degenerative changes in the muscles and skin leading to symmetric weakness and some degree of muscle atrophy, principally in the limb girdles, neck and pharynx. This disease has also shown to effect the esophagus, lungs, and least commonly the heart. Also classified in the umbrella term Inflammatory Myopathies.[2][3][4]
Epidemilogy[edit | edit source]
Female predilection. Bimodal age pattern: Juvenile dermatomyositis (JDM), affects children and is more severe; Adult dermatomyositis (ADM) usually affects adults around the age of 50.[1]
Characteristics/Clinical Presentation[edit | edit source]
Classically presents with a symmetrical proximal myopathy with associated dermatological changes including, dusky-red rash over the face, arms, hands, legs and other features. Other features include, dysphagia, myalgia, fever and weight loss[1]
Complications Malignancy[edit | edit source]
There is a sixfold increased risk of malignancy in dermatomyositis. This risk appears to be highest in patients diagnosed with dermatomyositis after 45 years of age. Risk factors include: >60 years old; male; dysphagia; necrosis of the skin; cutaneous vasculitis; accelerated onset of disease; increased creatine kinase (CK) levels; increased ESR and C-reactive protein (CRP) levels.[1]
Medications[5][6][edit | edit source]
Corticosteroids:
Immunosuppressive or cytotoxic agent:
- Methotrexate
- Azathioprine
- Cyclophosphamide
- Cyclosporin
- Mycophenolate mofetil
- Chlorambucil
Antimalarial: steroid-sparing agent to treat skin disease
- Hydroxychloroquine and chloroquine
Mainstay adequate antibody levels to improved muscle function and for skin lesions in patients whom corticosteroids and immunosupressives have failed:
- Intravenous immune globulin
Monoclonal antibodies (destruction of B cells):
- Rituximab
Calcium channel blocker: (gradual resolution of calcinosis)
Diagnostic Tests/Lab Tests/Lab Values[7][4][edit | edit source]
laboratory studies are helpful but nonspecific:
- ESR frequently elevated
- Antinuclear antibodies (ANA) elevated in 60 to 80 percent of patients
- Elevated creatine kinase (CK) enzyme, transaminases, and lactate dehyrogenase levels
- EMG to measure the electrical activity of the muscles: typically present as short-wave and fibrillations or myopathic pattern, 10 percent are false-negative
- Rheumatoid factor: elevated in 20 percent of patients, most often in those with overlap syndromes
- Neopterin and factor VIII–related antigen (von Willebrand factor): reported to correlate with juvenile DM
Causes[2][edit | edit source]
- The etiology of this disease is unknown
- Potential autoimmune mechanism; T cells inappropriately recognize muscle fiber antigens as foreign and attack the muscle tissues causing muscle breakdown
- Potentially triggered by a virus
- Potentially drug induced
Systemic Involvement[7][edit | edit source]
Systemic manifestations:
Common: proximal muscle weakness, dysphonia, dysphagia
Less common: respiratory muscle weakness, visual changes, abdominal pain
Systemic complications/association:
Cardiomyopathy
Cardiac conduction defects
Aspiration pneumonia secondary to respiratory muscle weakness
Diffuse interstitial pneumonitis/fibrosis
Large-bowel infarction secondary to vasculopathy has occurred in juvenile patients with myositis
Muscle atrophy
Muscle calcification
Ocular complications including iritis, nystagmus, cotton-wool spots, optic atrophy, conjunctival edema and pseudopolyposis
Internal malignancy
Medical Management (current best evidence)[4][7][edit | edit source]
The main goals when treating patients with dermatomyositis are improve function and prevent disability. The treatment regimen must be instituted early and requires a team approach between the physical therapist, dermatologist and family physician. While currently there is no cure for this systemic disease the symptoms can be treated. Corticosteroids are used initially to reduce the inflammation, shorten the time to normalization of muscle enzymes, and reduce morbidity. Maintenance therapy with Prednisone usually is necessary indefinitely in the adult patient. Immunosuppressive therapies may be used in individuals who do not respond well to the corticosteroids. The skin disease is primarily treated with sun avoidance, topical corticosteroids, antimalarial agents,methotrexate, mycophenolate mofetil, and/or intravenous immune globulin. Patient education is highly important to assist in control of the disease process.
Physical Therapy Management (current best evidence)[7][8][edit | edit source]
Preferred Practice Patterns for Physical Therapy:
- Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissue Dysfunction.
- Impairments/ Skin Involvement Into Fascia, Muscle, or Bone and Scar Formation
Focus of Treatment should include: Patient education on joint preservation
- Strengthening to prevent atrophy once inflammation is controlled
- Range of motion exercises to prevent contractures
- Passive stretching and splinting
Differential Diagnosis[6][edit | edit source]
- CREST Syndrome
- Parapsoriasis
- Graft Versus Host Disease
- Pityriasis Rubra Pilaris
- Lichen Myxedematosus
- Polymorphous Light Eruption
- Lichen Planus
- Psoriasis, Plaque
- Lupus Erythematosus, Acute
- Rosacea
- Lupus Erythematosus, Discoid
- Sarcoidosis
- Lupus Erythematosus, Subacute Cutaneous
- Tinea Corporis
- Morphea
- Urticaria, Chronic
- Multicentric Reticulohistiocytosis
Case Reports[edit | edit source]
Dermatomyositis: Evolution of a Diagnosis by Cleland J and Venzke J. Phys Ther (2003) 83: 932-945
http://www.intarchmed.com/content/1/1/25
http://pathhsw5m54.ucsf.edu/case34/case34.html
http://www.amc.edu/amr/archives/200201/case02.html
Resources[edit | edit source]
Support Groups:
Polymyositis and Dermatomyositis support group
General Information:
References[edit | edit source]
- ↑ 1.0 1.1 1.2 1.3 Radiopedia Dermatomyositis Available:https://radiopaedia.org/articles/dermatomyositis?lang=gb (accessed 23.1.2023)
- ↑ 2.0 2.1 Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. St. Louis, Missouri: Saunders Elsevier; 2009.
- ↑ MayoClinic.com Website. Dermatomyositis Available at: http://www.mayoclinic.com/health/dermatomyositis/DS00335 Accessed on 4/1/2010.
- ↑ 4.0 4.1 4.2 Hajj-ali RA. Polymyositis and Dermatomyositis. The Merck Manual of Diagnosis and Therapy. http://www.merck.com/mmpe/sec04/ch032/ch032d.html?qt=dermatomyositis&alt=sh. Updated February 2008. Accessed March 6, 2010.
- ↑ 5.0 5.1 Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL. Harrison's Manual of Medicine. 16th ed. McGraw-Hill, 2005
- ↑ 6.0 6.1 6.2 Callen JP. Dermatomyositis. http://emedicine.medscape.com/article/1064945-overview. Updated April 13,2009. Accessed March 10,2010
- ↑ 7.0 7.1 7.2 7.3 Koler RA, Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-72. http://www.aafp.org/afp/2001/1101/p1565.html. (accessed April 11, 2010)
- ↑ American Physical Therapy Association. Guide to Physical Therapy Practice. Phys Ther. 2001;81:9-744. Revised June 2003.