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<div class="noeditbox">Welcome to [[Pathophysiology of Complex Patient Problems|PT 635 Pathophysiology of Complex Patient Problems]] This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div><div class="editorbox">
[[Category:Rheumatology]]
'''Original Editors '''-Megan Saranson from [[Pathophysiology of Complex Patient Problems|Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  
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== <ref name="Hajj-ali">Hajj-ali RA. Polymyositis and Dermatomyositis. The Merck Manual of Diagnosis and Therapy. http://www.merck.com/mmpe/sec04/ch032/ch032d.html?qt=dermatomyositis&amp;amp;amp;alt=sh. Updated February 2008. Accessed March 6, 2010.</ref><ref name="Goodman et al.">Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. St. Louis, Missouri: Saunders Elsevier; 2009.</ref><ref name="mayo">MayoClinic.com Website. Dermatomyositis Available at: http://www.mayoclinic.com/health/dermatomyositis/DS00335 Accessed on 4/1/2010.</ref>Definition/Description  ==
== Introduction ==
[[File:Dermatomyositis.jpeg|thumb|Erythematous papules overlying the MCP and IP joints]]
Dermatomyositis is an autoimmune inflammatory myositis, possibly related condition to polymyositis.<ref name=":0">Radiopedia [https://radiopaedia.org/articles/dermatomyositis?lang=gb Dermatomyositis] Available:https://radiopaedia.org/articles/dermatomyositis?lang=gb (accessed 23.1.2023)</ref> 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. &nbsp;This disease has also shown to effect the esophagus, lungs, and least commonly the heart. Also classified in the umbrella term [[Inflammatory Myopathies]].<ref name="Goodman et al.">Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. St. Louis, Missouri: Saunders Elsevier; 2009.</ref><ref name="mayo">MayoClinic.com Website. Dermatomyositis Available at: http://www.mayoclinic.com/health/dermatomyositis/DS00335 Accessed on 4/1/2010.</ref><ref name="Hajj-ali">Hajj-ali RA. Polymyositis and Dermatomyositis. The Merck Manual of Diagnosis and Therapy. http://www.merck.com/mmpe/sec04/ch032/ch032d.html?qt=dermatomyositis&amp;alt=sh. Updated February 2008. Accessed March 6, 2010.</ref>&nbsp;


A systemic connective tissue disease, found in the rheumatoid family, 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. &nbsp;This disease has also shown to effect the esophagus, lungs, and least commonly the heart.<br>  
== Epidemilogy ==
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.<ref name=":0" />


{{#ev:youtube|hHHGB6_ESdQ}}
== Etiology ==
The cause of dermatomyositis is unknown, however several genetic, immunologic, and environmental factors are implicated in this condition.


== <ref name="Hajj-ali" /><ref name="Goodman et al." />Prevalence  ==
Genetic: there are studies suggested that there are particular human leukocyte antigen (HLA) types considered a risk factor for dermatomyositis<ref>O'Hanlon TP, Rider LG, Mamyrova G, Targoff IN, Arnett FC, Reveille JD, Carrington M, Gao X, Oddis CV, Morel PA, Malley JD. HLA polymorphisms in African Americans with idiopathic inflammatory myopathy: allelic profiles distinguish patients with different clinical phenotypes and myositis autoantibodies. Arthritis & Rheumatism: Official Journal of the American College of Rheumatology. 2006 Nov;54(11):3670-81.</ref><ref>Chinoy H, Salway F, Fertig N, Shepherd N, Tait BD, Thomson W, Isenberg DA, Oddis CV, Silman AJ, Ollier WE, Cooper RG. The UK Adult Onset Myositis Immunogenetic Collaboration (AOMIC): In adult onset myositis, the presence of interstitial lung disease and myositis specific/associated antibodies are governed by HLA class II haplotype rather than by myositis sub type. Arthritis Research and Therapy. 2005;8(1):R13.</ref>. In addition autoantibodies were also detected in patients.


• In the United States, approximately 5-10 in 1 million people are affected and the incidence appears to be increasing.<br>• May appear at any age but most commonly from age 40-60 in adulthood; in childhood 5-15<br>• The female to male ratio is 2:1<br><br>
Infection''':''' Viruses like Coxsackie B, enterovirus, and parvovirus are suspected triggers for dermatomyositis. They may initiate autoimmunity through mechanisms such as altering cellular proteins, disrupting self-tolerance, revealing hidden immune targets, activating B cells with autoantibodies, and molecular mimicry<ref>Adler BL, Cristopher-Stine L. Triggers of inflammatory myopathy: insights into pathogenesis. Discovery medicine. 2018 Feb;25(136):75.</ref>'''.'''


== <ref name="Callen" /><ref name="Goodman et al." />Characteristics/Clinical Presentation  ==
Certain drugs that including antineoplastic medications like hydroxyurea and cyclophosphamide, anti-infectious agents such as penicillin, sulfonamides, and isoniazid, [[NSAIDs|non-steroidal anti-inflammatory drugs]] like diclofenac and phenylbutazone, as well as D-penicillamine, statins, and certain vaccines can trigger dermatomyositis<ref>Dourmishev AL, Dourmishev LA. Dermatomyositis and drugs. Rheumaderm: Current Issues in Rheumatology and Dermatology. 1999 Jan 1:187-91.</ref>.


*Symptom onset maybe acute or insidious
== Characteristics/Clinical Presentation ==
*&nbsp;Progressive symmetric muscle weakness primarily in muscles of proximal joints and neck and pharynx
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" />.
*&nbsp;Dusky or erythematous skin rash, potentially scaly, elevated, or smooth. Typically found on the face resembling the butterfly rash associated with SLE, neck, shoulders, chest and back, forearms, lower legs, medial malleoli, and dorsum of the proximal interphalangeal and metacarpophalangeal joints.
[[File:Heliotrope.jpg|thumb|Heliotrope rash]]
* Muscle weakness: that starts slowly and worsens over time.  It first affects proximal muscles symmetrically, with distal muscles becoming involved later. This results in difficulties with everyday activities like standing up, climbing stairs, or lifting objects. Severe cases can lead to head drop, difficulty swallowing and speaking, and weakened breathing muscles. While less common, some patients may also experience muscle pain and tenderness.[[File:V-sign rash. Irregular patchy erythema.jpg|thumb|V-sign rash. Irregular patchy erythema.]]
* Skin disease: dermatomyositis (DM) is characterized by a rash it can appear before, alongside, or after muscle weakness the key skin features may include: <ref>Sontheimer RD. Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatologic clinics. 2002 Jul 1;20(3):387-408.</ref>
** Gottron's papules: Erythematous to violaceous bumps on the knuckles, potentially with scaling and ulceration, leading to scarring.
** Gottron's sign: Erythematous patches on elbows and knees (less specific).
** Heliotrope rash: Purple eyelid erythema with swelling and tiny blood vessels (telangiectasia), sometimes subtle in darker skin.
** Erythematous patches in sun-exposed and non-sun-exposed areas.
** Malar erythema: Redness across cheeks, extending to the nose and nasolabial folds.
** "V" sign: Confluent redness on the lower neck and upper chest.
** Shawl sign: Erythema on the upper back, neck, and shoulders, possibly extending to the arms.
** Scalp involvement and skin issues on the lower back and lateral thighs (Holster sign).
** Rarely, extensive erythroderma covering more than 50% of the body surface or a severe, cracked skin variant can occur.


[[Image:Rash.jpg]]&nbsp;
=== Systemic Involvement ===
 
'''Systemic manifestations:'''<br>Common: proximal muscle weakness, dysphonia, dysphagia<br>Less common: respiratory muscle weakness, visual changes, abdominal pain
*Gottron's papules&nbsp;
 
[[Image:Grotton papules- dermatomyositis.jpg|Image:Grotton_papules-_dermatomyositis.jpg]]
 
*&nbsp;Base and sides of nails maybe hyperemic
*&nbsp;Muscular pain and tenderness typically associated with the rash
*&nbsp;Subcutaneous calcifications may occur particularly in childhood
*diffuse alopecia with scaly scalp<br>
*&nbsp;Fatigue, fever, weight loss
*&nbsp;Difficulty swallowing
*&nbsp;Cardiopulmonary involvement such as arrhythmias, congestive heart failure, conduction deficits, ventricular hypertrophy, or pericarditis and respiratory muscles in severe cases
 
<br>
 
&nbsp;<br>
 
== <ref name="koler" />Subtypes&nbsp;<br>  ==
 
'''JUVENILE DERMATOMYOSITIS'''<br>While the clinical presentation of juvenile dermatomyositis is usually different from the presentation of the adult type, the skin lesions are similar, with the exception of an increased incidence of calcinosis cutis in juvenile patients. Common findings include low-grade fever, increased risk of gastrointestinal manifestations, and symmetric arthritis of the large and small joints. Asymptomatic cardiac conduction delays or right bundle branch block may be found in 50 percent of this group.<ref>Sullivan  DB, Cassidy  JT, Petty  RE.  Dermatomyositis in the pediatric patient.  Arthritis Rheum.  1977;20(suppl 2):327–31.</ref><ref>Pachman  LM.  Juvenile dermatomyositis.  Pediatr Clin North Am.  1995;42:1071–98.</ref>
 
Patients may exhibit weakness of the truncal muscles that requires them to use their arms to push themselves up from a prone position (i.e., Gower's sign). There does not appear to be any association between juvenile dermatomyositis and malignancy.<ref name="Kovacs">Kovacs  SO, Kovacs  SC.  Dermatomyositis.  J Am Acad Dermatol.  1998;39:899–920</ref>
 
'''OVERLAP SYNDROME'''<br>A number of patients with dermatomyositis also meet the criteria for one of the connective tissue disorders. To be a true overlap syndrome, the patient must meet the diagnostic criteria for each separate disorder. Overlap syndrome occurs more frequently in females than in males, with a 9:1 ratio.<ref name="Kovacs" /> Eleven to 40 percent of patients with dermatomyositis have been reported to have a concomitant diagnosis of a connective tissue disorder.<ref name="Kovacs" />


These disorders include '''rheumatoid arthritis, scleroderma, systemic lupus erythematosus, Sjögren's syndrome, polyarteritis nodosa and mixed connective tissue disease''', and these patients may present with'''''<i>p'</i>'''olyarthritis, sicca syndrome, sclerodactyly, Raynaud's phenomenon and late symptoms of myositis'''. Patients are also more likely to have positive nonmyositis–associated antibodies (such as double-stranded DNA, antinuclear antibodies [ANA], Scl-70, Jo-1 precipitating antibodies, PM-Scl, Ku antibodies or extractable nuclear antigen antibodies). ANA are found in up to 80 percent of patients with dermatomyositis or polymyositis, but this finding does not aid in distinguishing myositis from scleroderma or other rheumatologic diseases. Precipitating autoantibodies to the Mi-2 antigen are specific for dermatomyositis but are found in only about 20 percent of patients with dermatomyositis. In patients with overlap syndrome, the myositis tends to respond better to treatment with corticosteroids than it does in patients with an idiopathic etiology.
'''Systemic complications/association:'''


'''AMYOPATHIC DERMATOMYOSITIS'''<br>This classification has been controversial because it does not strictly meet the criteria put forth by Bohen and Peter.Amyopathic patients essentially have pathognomonic skin changes without clinical or laboratory evidence of muscle involvement. This condition has been reported in approximately 2 to 11 percent of patients with dermatomyositis. Patients most commonly present with lethargy, pruritus, fatigue, photosensitivity or arthralgias. In some cases, myositis developed later; in others, myositis that was not found by standard methods was suspected on the basis of magnetic resonance imaging (MRI).  
Aspiration pneumonia secondary to respiratory muscle weakness<br>Diffuse interstitial pneumonitis/fibrosis<br>Large-bowel infarction secondary to vasculopathy has occurred in juvenile patients with myositis<br>Muscle atrophy<br>Muscle calcification<br>Ocular complications including iritis, [[nystagmus]], cotton-wool spots, [[Optic Nerve|optic]] atrophy, conjunctival [[Oedema Assessment|edema]] and pseudopolyposis<br>Internal malignancy<br>[http://www.mayoclinic.com/health/cardiomyopathy/DS00519 Cardiomyopathy] Cardiac conduction defects<ref name="koler" />
== Complications '''Malignancy''' ==
There is a six fold 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>Zahr ZA, Baer AN. Malignancy in myositis. Curr Rheumatol Rep. 2011;13:208–15</ref>.


'''DERMATOMYOSITIS/MALIGNANCY'''<br>Although an increased risk of malignancy has not been associated with juvenile dermatomyositis, it has been demonstrated in adults with dermatomyositis. This risk appears to be highest in patients diagnosed with dermatomyositis after 45 years of age. The '''most commonly reported malignancies are ovarian and gastric cancer, and lymphoma'''. Other reported malignancies include lung, male genital organ, nonmelanoma skin, Kaposi's sarcoma, mycosis fungoides and melanoma.
== Diagnostic Tests/Lab Tests/Lab Values ==


Skin changes are not different in patients with or without malignancy. Therefore, careful investigation for malignancy should be initiated at the time dermatomyositis is diagnosed. In women with dermatomyositis, there is a significant association with ovarian cancer, and some authors recommend that the work-up for dermatomyositis include a comprehensive gynecologic evaluation, including a cancer antigen (CA-125) baseline screen, mammography and transvaginal ultrasonographic evaluation of the ovaries at baseline, and gynecologic examinations at six- to 12-month intervals for at least two years.
laboratory studies are helpful but nonspecific:


== <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" />Medications  ==
*&nbsp;ESR frequently elevated
*&nbsp;Antinuclear antibodies (ANA)&nbsp;elevated in 60 to 80 percent of patients
*&nbsp;Elevated creatine kinase (CK) enzyme, transaminases, and lactate dehyrogenase levels
*&nbsp;EMG to measure the electrical activity of the muscles: typically present as short-wave and fibrillations or&nbsp;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<ref name="koler">Koler RA, Montemarano A. [https://www.aafp.org/pubs/afp/issues/2001/1101/p1565.html Dermatomyositis]. American family physician. 2001 Nov 1;64(9):1565-73.</ref><ref name="Hajj-ali" />
== Management ==
The goals of managing dermatomyositis are focused on treating muscle weakness, skin disease, and addressing any other underlying complications. Physical therapy and rehabilitation play an essential role in management. <ref name=":1">Qudsiya Z, Waseem M. https://www.ncbi.nlm.nih.gov/books/NBK558917/ Available:https://www.ncbi.nlm.nih.gov/books/NBK558917/ (accessed 23.1.2023)</ref> 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:
=== Pharmacological Management ===
Corticosteroids are used initially to reduce the inflammation, shorten the time to normalization of muscle enzymes, and reduce morbidity.


*[http://www.drugs.com/prednisone.html Prednisone]
Maintenance therapy with Prednisone usually is necessary indefinitely in the adult patient.


Immunosuppressive or cytotoxic agent:&nbsp;
Immunosuppressive therapies may be used in individuals who do not respond well to the corticosteroids.


*Methotrexate
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.<ref name="koler" />
*Azathioprine
*Cyclophosphamide
*Cyclosporin
*Mycophenolate mofetil  
*Chlorambucil


Antimalarial: steroid-sparing agent to treat skin disease
=== Physical Therapy Management ===
Patients with mild disease should be encouraged to engage in active exercise programs. Range of motion exercises help in preventing contractures. Patients with esophageal dysfunction may require elevation of the head off the bed, thickening of feeds, and even feeding via gastric tubes when indicated.<ref name=":1" />


*Hydroxychloroquine and chloroquine
'''Focus of Treatment should include:&nbsp;'''  
 
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)
 
*Diltiazem
 
== <ref name="koler" /><ref name="Hajj-ali" />Diagnostic Tests/Lab Tests/Lab Values  ==
 
laboratory studies are helpful but nonspecific:<br>
 
*&nbsp;ESR frequently elevated
*&nbsp;Antinuclear antibodies (ANA)&nbsp;elevated in 60 to 80 percent of patients
*&nbsp;Elevated creatine kinase (CK) enzyme, transaminases, and lactate dehyrogenase levels
*&nbsp;EMG to measure the electrical activity of the muscles: typically present as short-wave and fibrillations or&nbsp;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<br>
 
== <ref name="Goodman et al." />Causes  ==
 
• The etiology of this disease is unknown<br>• Potential autoimmune mechanism; T cells inappropriately recognize muscle fiber antigens as foreign and attack the &nbsp; &nbsp;muscle tissues causing muscle breakdown<br>• Potentially triggered by a virus<br>• Potentially drug induced<br><br>
 
== <ref name="koler">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)</ref>Systemic Involvement  ==
 
'''Systemic manifestations:'''<br>Common: proximal muscle weakness, dysphonia, dysphagia<br>Less common: respiratory muscle weakness, visual changes, abdominal pain
 
'''Systemic complications/association:'''
 
[http://www.mayoclinic.com/health/cardiomyopathy/DS00519 Cardiomyopathy]<br>Cardiac conduction defects
 
Aspiration pneumonia secondary to respiratory muscle weakness<br>Diffuse interstitial pneumonitis/fibrosis<br>Large-bowel infarction secondary to vasculopathy has occurred in juvenile patients with myositis<br>Muscle atrophy<br>Muscle calcification<br>Ocular complications including iritis, nystagmus, cotton-wool spots, optic atrophy, conjunctival edema and pseudopolyposis<br>Internal malignancy<br>
 
== <ref name="Hajj-ali " /><ref name="koler" />Medical Management (current best evidence)  ==
 
&nbsp;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. &nbsp;While currently there is no cure for this systemic disease the symptoms can be treated. &nbsp;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. &nbsp;The skin disease is primarily treated with sun avoidance, topical corticosteroids, antimalarial agents,methotrexate, mycophenolate mofetil, and/or intravenous immune globulin. &nbsp;Patient education is highly important to assist in control of the disease process.<br>
 
== <ref name="koler" /><ref name="PT">American Physical Therapy Association. Guide to Physical Therapy Practice. Phys Ther. 2001;81:9-744. Revised June 2003.</ref>Physical Therapy Management (current best evidence)  ==
 
'''Preferred Practice Patterns for Physical Therapy:'''<br>4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissue &nbsp; &nbsp; &nbsp; Dysfunction.
 
7E: Impairments/ Skin Involvement Into Fascia, Muscle, or Bone and Scar Formation
 
'''Focus of Treatment should include:&nbsp;''' Patient education on joint preservation


*Strengthening to prevent atrophy once inflammation is controlled  
*Strengthening to prevent atrophy once inflammation is controlled  
*Range of motion exercises to prevent contractures  
*Range of motion exercises to prevent contractures  
*Passive stretching and splinting<br>
*Passive stretching and splinting<ref name="koler" /><ref name="PT">Alexandria V. American physical therapy association guide to physical therapist practice. Phys. Ther.. 1997;77(11):1163-74.</ref>


== Alternative/Holistic Management (current best evidence)  ==
== Differential Diagnosis  ==


'''Stress management: &nbsp;
*[http://emedicine.medscape.com/article/1064663-overview CREST Syndrome]  
 
*Meditation
*Yoga
 
'''Diet Changes:'''
 
**diet high in fiber (if large amount of muscle damage has occurred)
*low-fat
 
== <ref name="Callen">Callen JP. Dermatomyositis. http://emedicine.medscape.com/article/1064945-overview. Updated April 13,2009. Accessed March 10,2010</ref>Differential Diagnosis  ==
 
*[http://emedicine.medscape.com/article/1064663-overview CREST Syndrome]
*Parapsoriasis  
*Parapsoriasis  
*Graft Versus Host Disease  
*Graft Versus Host Disease  
Line 147: Line 85:
*Polymorphous Light Eruption  
*Polymorphous Light Eruption  
*Lichen Planus  
*Lichen Planus  
*Psoriasis, Plaque  
*[[Psoriatic Arthritis|Psoriasis]], Plaque  
*[http://emedicine.medscape.com/article/1065292-overview Lupus Erythematosus, Acute]
*[http://emedicine.medscape.com/article/1065292-overview Lupus Erythematosus, Acute]  
*Rosacea  
*Rosacea  
*Lupus Erythematosus, Discoid  
*Lupus Erythematosus, Discoid  
*Sarcoidosis  
*[[Sarcoidosis]]
*Lupus Erythematosus, Subacute Cutaneous  
*[[Systemic Lupus Erythematosus|Lupus Erythematosu]]<nowiki/>s, Subacute Cutaneous  
*Tinea Corporis  
*Tinea Corporis  
*Morphea  
*Morphea  
*Urticaria, Chronic  
*Urticaria, Chronic  
*Multicentric Reticulohistiocytosis<br>
*Multicentric Reticulohistiocytosis<br>
== Case Reports  ==
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 <br>  ==
Support Groups:
http://www.dailystrength.org/c/Polymyositis-and-Dermatomyositis/support-group<br>
http://www.myositissupportgroup.org/
General Information:
http://www.myositis.org/template/page.cfm?id=189
http://www.ninds.nih.gov/disorders/dermatomyositis/dermatomyositis.htm
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
see tutorial on [[Adding PubMed Feed|Adding PubMed Feed]]
<div class="researchbox"><rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1neszevTVzp28kncao7Amgni3j9nHjz76CjztpQxYq46oUWMJl|charset=UTF-8|short|max=10</rss></div>
== References  ==
== References  ==
see [[Adding References|adding references tutorial]].


<references />  
<references />  


[[Category:Bellarmine_Student_Project]]
[[Category:Bellarmine_Student_Project]] 
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Latest revision as of 22:32, 30 September 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]

Erythematous papules overlying the MCP and IP joints

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]

Etiology[edit | edit source]

The cause of dermatomyositis is unknown, however several genetic, immunologic, and environmental factors are implicated in this condition.

Genetic: there are studies suggested that there are particular human leukocyte antigen (HLA) types considered a risk factor for dermatomyositis[5][6]. In addition autoantibodies were also detected in patients.

Infection: Viruses like Coxsackie B, enterovirus, and parvovirus are suspected triggers for dermatomyositis. They may initiate autoimmunity through mechanisms such as altering cellular proteins, disrupting self-tolerance, revealing hidden immune targets, activating B cells with autoantibodies, and molecular mimicry[7].

Certain drugs that including antineoplastic medications like hydroxyurea and cyclophosphamide, anti-infectious agents such as penicillin, sulfonamides, and isoniazid, non-steroidal anti-inflammatory drugs like diclofenac and phenylbutazone, as well as D-penicillamine, statins, and certain vaccines can trigger dermatomyositis[8].

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].

Heliotrope rash
  • Muscle weakness: that starts slowly and worsens over time. It first affects proximal muscles symmetrically, with distal muscles becoming involved later. This results in difficulties with everyday activities like standing up, climbing stairs, or lifting objects. Severe cases can lead to head drop, difficulty swallowing and speaking, and weakened breathing muscles. While less common, some patients may also experience muscle pain and tenderness.
    V-sign rash. Irregular patchy erythema.
  • Skin disease: dermatomyositis (DM) is characterized by a rash it can appear before, alongside, or after muscle weakness the key skin features may include: [9]
    • Gottron's papules: Erythematous to violaceous bumps on the knuckles, potentially with scaling and ulceration, leading to scarring.
    • Gottron's sign: Erythematous patches on elbows and knees (less specific).
    • Heliotrope rash: Purple eyelid erythema with swelling and tiny blood vessels (telangiectasia), sometimes subtle in darker skin.
    • Erythematous patches in sun-exposed and non-sun-exposed areas.
    • Malar erythema: Redness across cheeks, extending to the nose and nasolabial folds.
    • "V" sign: Confluent redness on the lower neck and upper chest.
    • Shawl sign: Erythema on the upper back, neck, and shoulders, possibly extending to the arms.
    • Scalp involvement and skin issues on the lower back and lateral thighs (Holster sign).
    • Rarely, extensive erythroderma covering more than 50% of the body surface or a severe, cracked skin variant can occur.

Systemic Involvement[edit | edit source]

Systemic manifestations:
Common: proximal muscle weakness, dysphonia, dysphagia
Less common: respiratory muscle weakness, visual changes, abdominal pain

Systemic complications/association:

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
Cardiomyopathy Cardiac conduction defects[10]

Complications Malignancy[edit | edit source]

There is a six fold 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[11].

Diagnostic Tests/Lab Tests/Lab Values[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[10][4]

Management[edit | edit source]

The goals of managing dermatomyositis are focused on treating muscle weakness, skin disease, and addressing any other underlying complications. Physical therapy and rehabilitation play an essential role in management. [12] 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.

Pharmacological Management[edit | edit source]

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.[10]

Physical Therapy Management[edit | edit source]

Patients with mild disease should be encouraged to engage in active exercise programs. Range of motion exercises help in preventing contractures. Patients with esophageal dysfunction may require elevation of the head off the bed, thickening of feeds, and even feeding via gastric tubes when indicated.[12]

Focus of Treatment should include: 

  • Strengthening to prevent atrophy once inflammation is controlled
  • Range of motion exercises to prevent contractures
  • Passive stretching and splinting[10][13]

Differential Diagnosis[edit | edit source]

References[edit | edit source]

  1. ↑ 1.0 1.1 1.2 Radiopedia Dermatomyositis Available:https://radiopaedia.org/articles/dermatomyositis?lang=gb (accessed 23.1.2023)
  2. ↑ Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. St. Louis, Missouri: Saunders Elsevier; 2009.
  3. ↑ MayoClinic.com Website. Dermatomyositis Available at: http://www.mayoclinic.com/health/dermatomyositis/DS00335 Accessed on 4/1/2010.
  4. ↑ 4.0 4.1 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. ↑ O'Hanlon TP, Rider LG, Mamyrova G, Targoff IN, Arnett FC, Reveille JD, Carrington M, Gao X, Oddis CV, Morel PA, Malley JD. HLA polymorphisms in African Americans with idiopathic inflammatory myopathy: allelic profiles distinguish patients with different clinical phenotypes and myositis autoantibodies. Arthritis & Rheumatism: Official Journal of the American College of Rheumatology. 2006 Nov;54(11):3670-81.
  6. ↑ Chinoy H, Salway F, Fertig N, Shepherd N, Tait BD, Thomson W, Isenberg DA, Oddis CV, Silman AJ, Ollier WE, Cooper RG. The UK Adult Onset Myositis Immunogenetic Collaboration (AOMIC): In adult onset myositis, the presence of interstitial lung disease and myositis specific/associated antibodies are governed by HLA class II haplotype rather than by myositis sub type. Arthritis Research and Therapy. 2005;8(1):R13.
  7. ↑ Adler BL, Cristopher-Stine L. Triggers of inflammatory myopathy: insights into pathogenesis. Discovery medicine. 2018 Feb;25(136):75.
  8. ↑ Dourmishev AL, Dourmishev LA. Dermatomyositis and drugs. Rheumaderm: Current Issues in Rheumatology and Dermatology. 1999 Jan 1:187-91.
  9. ↑ Sontheimer RD. Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatologic clinics. 2002 Jul 1;20(3):387-408.
  10. ↑ 10.0 10.1 10.2 10.3 Koler RA, Montemarano A. Dermatomyositis. American family physician. 2001 Nov 1;64(9):1565-73.
  11. ↑ Zahr ZA, Baer AN. Malignancy in myositis. Curr Rheumatol Rep. 2011;13:208–15
  12. ↑ 12.0 12.1 Qudsiya Z, Waseem M. https://www.ncbi.nlm.nih.gov/books/NBK558917/ Available:https://www.ncbi.nlm.nih.gov/books/NBK558917/ (accessed 23.1.2023)
  13. ↑ Alexandria V. American physical therapy association guide to physical therapist practice. Phys. Ther.. 1997;77(11):1163-74.
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