Dermatomyositis Clinical Presentation

  • Author: Jeffrey P Callen, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Oct 12, 2011
 

History

Persons with dermatomyositis often present with skin disease as one of the initial manifestations. In perhaps as many as 40% of individuals with dermatomyositis, the skin disease is the sole manifestation at onset. Muscle disease may occur concurrently, may precede the skin disease, or may follow the skin disease by weeks to years.

Muscle involvement manifests as proximal muscle weakness. Affected patients often begin to note muscle fatigue or weakness when climbing stairs, walking, rising from a sitting position, combing their hair, or reaching for items in cabinets that are above their shoulders. Muscle tenderness may occur but is not a regular feature of dermatomyositis.

Systemic manifestations may occur; therefore, the review of systems should assess for the presence of arthralgia, arthritis, dyspnea, dysphagia, arrhythmia, and dysphonia.

Malignancy is possible in any patient with dermatomyositis, but it is much more common in adults older than 60 years. Only a handful of children with dermatomyositis and malignancy have been reported. The history should include a thorough review of systems, as well as an assessment for previous malignancy.

Dermatomyositis occurs in children as well as in adults. It is characterized by muscle weakness. Dermatomyositis in children resembles the adult form. Children commonly develop a tiptoe gait secondary to flexion contracture of the ankles in early childhood. Children tend to have extramuscular manifestations, especially gastrointestinal (GI) ulcers and infections, more frequently than adults do. Extramuscular manifestations of the disease may include the following:

  • General systemic disturbances, fever, arthralgia, malaise, weight loss, Raynaud phenomenon
  • Dysphagia, similar to that of scleroderma
  • Atrioventricular defects, tachyarrhythmias, dilated cardiomyopathies
  • GI ulcers and infections
  • Contracture of joints
  • Pulmonary involvement due to weakness of thoracic muscles, interstitial lung disease

Na et al found the frequency of subcutaneous calcifications to be significantly higher in juvenile dermatomyositis than adult dermatomyositis.[22]

Several reports describe drug-induced dermatomyositis or existing dermatomyositis exacerbated by certain drugs, including statins and interferon therapy.[23]

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Physical Examination

Dermatomyositis is a disease that primarily affects the skin and the muscles, but it might also affect other organ systems.

The characteristic and possibly pathognomonic cutaneous features of dermatomyositis are a heliotrope (ie, blue-purple) discoloration on the upper eyelids (see the first image below) and a raised, violaceous, scaly eruption on the knuckles (ie, Gottron papules; see the second image below).

Heliotrope rash in a woman with dermatomyositis. Heliotrope rash in a woman with dermatomyositis. Gottron papules and nailfold telangiectasia are prGottron papules and nailfold telangiectasia are present in this patient with dermatomyositis.

Characteristic but not pathognomonic features include malar erythema, poikiloderma in a photosensitive distribution, violaceous erythema on the extensor surfaces, and periungual and cuticular changes (see the images below).

Dermatomyositis is often associated with a poikiloDermatomyositis is often associated with a poikiloderma in a photodistribution. These lesions on dorsal hands demonstrate photodisThese lesions on dorsal hands demonstrate photodistribution of dermatomyositis. Note sparing of interdigital web spaces.

There is commonly a flat, red rash involving the face and upper trunk. The erythematous lesions may result in scaling, pigmentation, and depigmentation of the skin, producing a shiny appearance. The rash may involve other body surfaces, including knees, elbows, neck, anterior chest (ie, V sign), or back and shoulders (ie, shawl sign); sun exposure can exacerbate the rash.

Patients often notice an eruption on exposed surfaces. The disease is often pruritic, and, sometimes, intense pruritus may disturb sleep patterns. Patients may also complain of a scaly scalp or diffuse hair loss (see the image below).[24]

Diffuse alopecia with scaly scalp dermatosis is coDiffuse alopecia with scaly scalp dermatosis is common in patients with dermatomyositis.

Dilated capillary loops at the base of the fingernail are characteristic of dermatomyositis. The cuticles may be irregular and thickened, and the palmar and lateral surfaces of the fingers may become rough and cracked.

Children with dermatomyositis may have an insidious onset that hides the true diagnosis until the dermatologic disease is clearly observed and diagnosed. Calcinosis is a complication of juvenile dermatomyositis (see the image below), but it is rarely observed at the onset of disease. African Americans and patients in lower socioeconomic groups are more likely to have a delay in their diagnosis. The prognosis in children with dermatomyositis is worse in those in whom diagnosis is delayed.

Calcinosis caused by dermatomyositis in childhood Calcinosis caused by dermatomyositis in childhood can be observed in patient who had active dermatomyositis 15 years before time of this photograph.

Muscle disease commonly manifests as a proximal symmetrical muscle weakness. The degree of weakness may range from mild to moderate to severe; sometimes, quadriparesis is observed. Patients may have difficulty rising from a chair or squatting and then raising themselves from this position. Sometimes, in an effort to rise, patients use other muscles that are not as affected. Testing of muscle strength is part of each patient assessment. Often, the extensor muscles of the arms are more affected than the flexor muscles. Distal strength is almost always maintained.

Muscle pain and tenderness are observed early in the course of the disease; muscle tenderness is a variable finding. Sensation is normal, and tendon reflexes are preserved unless the muscle is severely weak and atrophic.

Other systemic features include joint swelling, changes associated with Raynaud phenomenon, and abnormal findings on cardiopulmonary examination. Joint swelling occurs in some patients with dermatomyositis. The small joints of the hands are the most frequently involved. The arthritis associated with dermatomyositis is nondeforming. Patients with pulmonary disease may have abnormal breath sounds. Patients with an associated malignancy may have physical findings relevant to the affected organs.

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Contributor Information and Disclosures
Author

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Additional Contributors

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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Heliotrope flower, for which characteristic manifestation of dermatomyositis is named.
Heliotrope rash in a woman with dermatomyositis.
Gottron papules and nailfold telangiectasia are present in this patient with dermatomyositis.
These lesions on dorsal hands demonstrate photodistribution of dermatomyositis. Note sparing of interdigital web spaces.
Diffuse alopecia with scaly scalp dermatosis is common in patients with dermatomyositis.
Dermatomyositis is often associated with a poikiloderma in a photodistribution.
Histopathology of dermatomyositis is interface dermatitis.
Calcinosis caused by dermatomyositis in childhood can be observed in patient who had active dermatomyositis 15 years before time of this photograph.
Histopathology of dermatomyositis showing inflammatory myopathic changes with a predominantly perivascular chronic inflammatory infiltrate.
Calcifying panniculitis in patient with dermatomyositis.
Ulceration over dorsal and lateral fingers in patient with dermatomyositis.
Hematoxylin and eosin paraffin section shows polymyositis. Longitudinal section shows dense, chronic, endomysial inflammatory infiltrate. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin frozen section shows polymyositis. Endomysial chronic inflammation is present among intact myofibers that are remarkable only for increased variability of fiber size. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin section shows polymyositis. Patient had dense endomysial inflammation that contains abundance of plasma cells, which can be observed in patients with chronic polymyositis. Two necrotic myofibers, characterized by dense eosinophilic staining, are observed. Focal fatty infiltration of muscle is present in lower left quadrant of photomicrograph. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin section shows polymyositis. Photomicrograph illustrates attack on nonnecrotic myofiber by autoaggressive T lymphocytes. On left, central myofiber is intact. On right, it is obliterated by segmental inflammatory attack. If immunohistochemistry were performed, expected findings would include admixture of CD8 T lymphocytes and macrophages in inflammatory process. Image courtesy of Roberta J Seidman, MD.
Hematoxylin and eosin paraffin shows dermatomyositis. In dermatomyositis, inflammation is characteristically perivascular and perimysial. Vessel oriented approximately vertically in center has mild perivascular chronic inflammatory infiltrate. Endothelium is plump; wall is not necrotic. A few lymphocytes in wall of vessel are probably in transit from lumen to external aspect of vessel. Some observers may interpret this finding as vasculitis, but it is certainly neither necrotizing vasculitis nor arteritis. Image courtesy of Roberta J. Seidman, MD.
Hematoxylin and eosin frozen section shows perifascicular atrophy in dermatomyositis. Fascicles in this sample show atrophy, predominantly at periphery, along connective-tissue border. Ischemia is considered to cause perifascicular atrophy. This finding is characteristic of dermatomyositis, mostly associated with juvenile form but also observed in adult form. Image courtesy of Roberta J Seidman, MD.
Immunofluorescence for membrane attack complex of complement (MAC) in dermatomyositis. Bright ring of yellow-green fluorescence at center represents MAC in wall of microvessel. Finding was not present after treatment with steroids. Image courtesy of Roberta J Seidman, MD.
 
 
 
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